Incontinentia pigmenti (IP) is a rare genetic multisystem disorder that may 
affect many organs including the skin, bone, eyes and the central nervous 
system. Central nervous system manifestations are seen in 30% of cases with 
seizures and mental retardation. Seizures occurring as the presenting sign of IP 
are rarely reported. We report a case of a female newborn with IP who had 
seizures on day 4 of life, which were followed in her second month by the 
development of the characteristic cutaneous changes for IP. With this case 
report, we would like to emphasize the need for inclusion of IP in the 
differential diagnosis of neonatal seizures.