A case of multiple sebaceous adenomas associated with internal malignant disease is further documented. Thirteen similar cases reported in the literature are also reviewed. We support the thesis that this association represents a unique syndrome. It can be seen in both sexes and usually is manifested during the fifth and sixth decades. A family history of malignant disease was revealed among seven of the nine cases mentioned. Of the other skin lesions, keratoacanthoma is also frequently seen. The internal malignant tumors most commonly afflict the gastrointestinal tract, especially the large intestine. With proper treatment the internal tumors appear to be of a low degree of malignancy. However, these patients have a propensity to develop muliple visceral tumors and require periodic follow-up. Some patients develop skin tumors prior to the discovery of internal malignant disease, an event that offers some prognostic value. Chromosomal study of the cultured blood lymphocytes by the Giemsa banding technique failed to reveal any abnormality.