BACKGROUND: Desmoid tumor is a rare soft tissues neoplasia characterized by local invasiveness and by a tendency towards local recurrence although not towards metastasization. DISCUSSION: Etiology is not clear. Desmoid tumors originate from the monoclonal proliferation of one mesenchymal cell and develop in the context of fascial, muscular and aponeurotic tissue. They are free of capsule and do not usually metastatize, although they do present a high risk of local recurrence CONCLUSION: In the absence of a systematic data collection and of a clear distinction, in the existing databases, between primitive and recurrent lesions and between lesions of different sites, there are no standard guidelines for a correct management of desmoids.