Cervical lipomas are tumors that may present as painless masses with slow growth. This type of tumors is usually asymptomatic until they reach a large size. They can be solitary or multiple. When multiple and symmetrical distributed around neck area, the lesions can be classified as Madelung's disease, a rare condition that affects mostly Mediterranean men. Our objective was to describe the symptoms, cytological and histological aspects, diagnostic problems and treatment strategies of the large size lipomas and to highlights that the differential diagnosis between common lipoma of cervical area and Madelung's disease can be established only on clinical grounds, with major implications in the treatment options.