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Background: Colloid cysts of third ventricle are rare lesions which may present with symptoms of acute hydrocephalus. We report a case of acute obstructive hydrocephalus secondary to third ventricular colloid cyst and its management during 1st trimester pregnancy. Case Description: A 31-years-old lady presented to the emergency department with reduced consciousness. Computed tomography head showed obstructive hydrocephalus and an obstructing lesion near foramen of Monro suggestive of third ventricle colloid cyst. She underwent endoscopic resection of colloid cyst. Her pregnancy was only confirmed after surgery and she delivered a healthy neonate at full term. She has remained clinically well and had resumed her work as a general practitioner. Conclusion: This case illustrates that urinary pregnancy test may show false negative result but pregnancy should not preclude radiological investigation and neurosurgical intervention in patients with deteriorating neurological function.