Abstract
A newborn with a diffuse epidermal nevus and with neurological symptoms had a dramatic clinical course and died at 36 hours of age. Necropsy findings included leptomeningeal hemangioma, voluminous cystic adenoma of the liver, horseshoe kidney, patent ductus arteriosus, and lipodermoids of the conjunctivae. Although there was no sebaceous gland involvement, a diagnosis of linear nevus sebaceous syndrome was made. Of the cases of this syndrome reported to date, ours is the most severe, and the first case, to our knowledge, in which it has been possible to perform a complete anatomical study.
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