A female newborn presented with emerging skin lesions, systemic eosinophilia, and eosinophilic reaction in the skin, liver, lungs, spleen, lymphatic nodes, porencephalia, convulsions, and disorders of thermoregulation. In addition to that, respiratory and heart failure, as well as brain edema were progressing. The suspected diagnosis of incontinentia pigmenti (Bloch-Sulzberger syndrome) was confirmed postmortem by skin biopsy.