Incontinentia pigmenti (IP) is a rare X linked dominant genetic multisystem neurocutaneous disorder that may affect many organs including the skin, bone, eyes and the central nervous system. Central nervous system manifestations are seen in 30%-40% of cases with seizures and mental retardation. Seizures as the presenting sign of IP in neonates are rarely reported. We report a case of a female newborn with IP who had pleomorphic skin rashes and erythematous vesicles over upper and lower extremities since 5th day of life which were followed by seizures on day 22nd of life. With this case report, we would like to emphasize the need for inclusion of IP in the differential diagnosis of neonatal seizures and to confirm diagnosis of all such unusual skin lesions histologically.