Dermatomyositis and carcinoma of colon were diagnosed in a 66-year-old woman. Meticulous physical examination excluded further systemic or cutaneous involvement. The musculocutaneous disorders responded well to daily oral corticosteroid, and the malignant tumor was totally removed surgically. After a seven-year follow-up of actual dermatomyositis controlled by maintenance doses of prednisone ranging from 5 to 15 mg daily, the patient developed a meningioma. Current concepts and data regarding various aspects of the combination between dermatomyositis and tumors are discussed. To our knowledge, this is the first reported case of meningioma associated with dermatomyositis.