Numerous skin lesions: sebaceous hyperplasia, benign acanthoma, keratoacanthoma and squamous cell epithelioma appeared on the face, especially on the nose, cheeks and around the lips of a 45 yr old abese man with mild diabetes. In 1969, an adenocarcinoma was found in the rectosigmoid and another one in the colon. In 1974, excision of a well differentiated epidermoid carcinoma was performed in the right external auditory meatus. A rectoscopy showed a micropolyp at the rectosigmoid junction. In 1975, an abdominal exploration was performed following a sudden relapse of about 50 skin lesions in the face, partly sebaceous adenomas, partly sebaceous gland epitheliomas and about 10 precancerous lesions on the dorsum of the hands. A villous adenoma of the colon was removed. Inheritance appears to be autosomal dominant with variability in the expressiveness of the cutaneointestinal symptoms.