Madelung-Launois-Bensaude (MLB) disease is uncommon and probably under-diagnosed. It is defined as a symmetrical proximal accumulation of fat. Its physiopathology is obscure, but favouring factors, like alcohol or corticosteroids, are well known. We describe, for the first time, an unusual case of distal form of the disease, involving hands and feet simultaneously. Moreover, this phenotype was associated with a proximal form of MLB, and new localizations in the elbows and knees have been identified. We review the literature concerning distal forms of MLB, and compare the features. This disease is an interesting new differential diagnosis of acromelic arthritis.