Here we are describing a case of neurofibromatosis1(NF1) with facial plexiform neurofibroma presenting as bilateral orbital ptosis. The patient had cafe au lait spots, freckling and plexiform neurofibroma. To the best of our knowledge only one case was reported in literature with bilateral ptosis in plexiform neurofibroma without any systemic features of NF1 from Ireland. No case has been reported with similar condition from India. Introduction: Neurofibromatosis I (Van Recklinghausen disease) is an autosomal dominant disease with incomplete penetrance and variable expression.Its incidence is about 1:3000 live births. Diagnostic criteria include: 1) Six or more cafe-au-lait spots, >5 mm in size in prepubertal and > 15 mm in postpuberal age group; 2) Two or more neurofibromas of any type or one plexiform neurofibroma; 3) Freckling of the axillary or inguinal regions (Crowes sign), 4) Optic glioma; 5) Two or more Lisch nodules; 6) Osseous lesions such as sphenoid dysplasia or thinning of cortex of long bones (with or without pseudoarthrosis); 7) First-degree relative (parent, sibling, offspring) with neurofibromatosisI. Diagnosis is made if two or more criteria are present[1].Plexiform neurofibroma though among one of the criteria of NF1, is itself very significant because of its tendency to grow, turn malignant and producing cosmetic as well as functional problems[2]. Case: A 14 year old male child presented to us with bilateral ptosis and not able to open eyes leading to visual handicap. He was IUGR with birth weight of 1.5 kg and had one month NICU stay. Since then parents noticed swelling over left half of the face involving left upper and lower eyelids.This swelling was growing keeping pace with the growth of the child. There was also very small swelling over right upper eyelid. He also had global developmental delay with all milestones achieved one to one and half year later than normal child. Patient had no history of headache, convulsion and hearing impairment. No other family member was having NF1. Figure 1 A: Clinical picture of patient showing plexiform neurofibroma with bilateral ptosis B:Cafe au lait spots and freckling on the back of patient On examination at present the patient was malnourished and stunted with no sign of puberty. General examination shows swelling over left half of face along with both eyelids, freckling over groin, axilla and whole trunk. Four big Cafe au lait spots measuring 6x4, 3x1, 5x3,3x2 cm size and multiple small cafe au lait spots of 0.5 to 1 cm were present at various parts of the body. The swelling of face was extending from left half of nose medially to left pinna laterally, superiorly involving left upper and lower eyelids, right upper eyelid and upper lip, causing disfigurement. The margins of swelling were indistinct, consistency was soft like “bag of worms” as described in literature and overlying skin was hyperpigmented. There was also dimple in the left pinna. There was malocclusion of teeth and persistence of deciduous teeth. There were no neurofibromas, lisch nodules or any bone deformity. MRI of patient was done which revealed moderate supratentorial hydrocephalous. Rest of brain parenchyma was normal. Sella was enlarged with non-visualization of anterior clinoid processes. Bilateral optic nerves were vertically tortutous but no evidence of tumor was present. There was evidence of retinal detachment of right eye. Ipsilateral lens also appears deformed and displaced. Ill-defined soft tissue thickening was also noted involving bilateral upper eyelids (right side> left side), left cheek, nasal vestibule and upper lip. A relatively defined nodular thickening was also noted in left masticular space. Patient underwent ptosis-correction surgery in both the eyes, retinal detachment surgery and lensectomy of the right eye. Since parents were not concerned for asthesis and no major functional impairment was present, so further treatment was deffered and decision to keep patient under regular six monthly follow up was taken. Histopathology of right lid swelling showed a firm nodule measuring 2.5x1x0.5 cm. Hematoxylin and Eosin sections showed features of a plexiform neurofibroma. An epidermoid cyst with heavy infiltration by chronic inflammation was also seen in surrounding soft tissue. No evidence of malignancy seen in the sections studied. This patient fulfilled three criteria for NF1and probably developed disease due to spontaneous mutation.