ABSTRACT
Congenital cytomegalovirus (CMV) infection is one of the most frequent congenital infections in neonates. It can manifest as asymptomatic infection in 90% or symptoms may appear in 10% of the patients. Asymptomatic congenital CMV infection is likely to be a leading cause of sensorineural hearing loss, mental retardation and microcephaly. Incontinentia pigmenti is a rare multisystemic ectodermal disorder, which is characterized by vesicular, verrucous, and pigmented cutaneous lesions, and is frequently associated with various developmental defects of the eyes, CNS, teeth, hair, and nail. It is regarded as an X-linked dominent genetic disorder. We are reporting a case of neonate who presented with delayed development and bilateral hearing loss due congenital CMV infection which was incidentally assoicated with incontinentia pigmenti.