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Sasi Bhushan Gottimukkala, Madhu Jagalasar, Giridhar Sethuraman, Srinivasan Kitchanan Department of Neonatology, Chettinad Hospital & Research Institute, Chennai, Tamil Nadu, India Abstract: Incontinentia pigmenti is a rare X-linked dominant multi-system disorder that is clinically suspected, based on the characteristic evolution of skin lesions through four stages. We describe a term neonate who presented at birth with pleomorphic skin rashes, including linear hyperpigmented hyperkeratotic lesions and erythematous vesicles, and then developed seizures. She was later histologically and genetically confirmed as a case of incontinentia pigmenti. The simultaneous presence of three stages of skin lesions in the neonatal period is an atypical presentation, which has not been previously well described. Keywords: incontinentia pigmenti, IKBKG gene, seizures, infant, newborn