[Systematic sebaceous nevus with multiple secondary tumors as the chief symptom of Schimmelpenning-Feuerstein-Mims syndrome].

A women presented with a systematized sebaceous nevus of the head and neck. She had developed multiple basal cell carcinomas and sebaceomas in the nevus over the past 3 years after a period of about 55 years without any changes. Detailed medical history and physical examination revealed a constellation of findings suggesting a syndrome. Besides ptosis and a congenital, vascular corneal clouding at the right, there was hypoplasia of the right maxillary sinus, a right labyrinthine deafness and recurrent inguinal herniae on the right. The Schimmelpenning-Feuerstein-Mims-Syndrome was diagnosed.