Numerous skin lesions: sebaceous hyperplasias, benign acanthoma, keratoacanthomas and squamous cell epitheliomas appeared on the face, especially on the nose, on the cheeks and around the lips of a 45-year-old obese man with mild diabetes. In 1969, an adenocarcinoma had been found in the rectosigmoid and another one in the colon. In 1974, an excision of a well-differenciated 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 épithéliomas, and about 10 precancerous lesions on the dorsum of the hands. A villous adenoma of the colon was removed. Inheritance appears to the autosomal dominant with variability in the expressiveness of the cutaneo-intestinal symptom