The clinical case refers to a three months-old female patient with incontinentia pigmenti. The cutaneous lesions, were the predominant features of the dermatosis; it consisted in vesicles, bullae, hyperkeratosis and pigmentation forming bizarre patterns. Associated with the dermatosis there were neurological abnormalities, which consisted in cerebral ulegiria. The child died during an episode of bronchiolitis. The necropsy showed; ulegiria cerebral, severe bronchopneumonia lobar and the cutaneous lesions.