After a review of literature and the report of a case with intrathoracic location, the authors consider present criteria for a correct diagnosis of low grade soft tissue sarcoma, when the possibility of nodular fasciitis is under evaluation. Nodular fasciitis is a benign pathology up to day not well characterised from the clinical and pathological point of view. The authors conclude that differential diagnosis between low grade soft tissue sarcoma and nodular fasciitis is frequently possible only on the basis of clinical course evaluation; otherwise the management may be inadequate.