Familial lymphedema should be classified in 2 categories depending on onset. Milroy''s disease, or congenital hereditary lymphedema, is present from birth, painless, without tendency to ulcerate, and may have cholestasis or intestinal lymphangiectasia associated with it. Meige''s disease, hereditary lymphedema praecox, is lymphedema with onset in the 1st or 2nd decade, often presenting with inflammation, and may have a number of associated related anomalies including distichiasis, extradural cysts, vertebral anomalies, cerebrovascular malformation, yellow nails and sensorineural hearing loss. Both types follow an autosomal dominant pattern. A family of 39 persons in 5 generations with 13 affected persons having Meige''s disease is presented. The importance of recognizing this type of familial lymphedema is discussed.