The authors present a case of intraosseus lipoma seen at the Orthopaedic Clinic of the University of Bari, bringing the total in the world literature to thirty cases. The tumour is a benign primary neoplasm of bone, made up of mature adipose tissue containing atrophic bone trabeculae. This affection is a definite entity quite distinct from parosteal lipomas arising in periosteum, fasciae and muscles which only affect the bone secondarily by compression or direct invasion. Emphasizing the extreme rarity of the tumour, the authors discuss its clinical, radiological, anatomical and pathological features. The differential diagnosis is with certain dysplasic and neoplastic lesions of bone such as: bone cyst, non-ossifying fibroma, aneurismal cyst, monostotic fibrous dysplasia, and solitary myeloma. Complete removal is followed by cure.