Benign symmetric lipomatosis is a rare disease. Its main characteristic is the symmetric deposition of fat, in an unencapsulated form, at typical sites in the body, but primarily in the head and neck region. Its aetiopathogenesis is unknown. A disturbance of lipid metabolism is involved, and there is a proven connection with chronic alcoholism. Problems with the differential diagnosis of the characteristic clinical picture arise from the fact that this disease is not widely recognized. Over a period of 30 years, the authors treated 31 patients with benign symmetric lipomatosis. They review the pertinent literature and report retrospectively on the fates of the 11 surviving patients.