Objective: To explore the clinicopathologic features of desmoid-type fibromatosis (DTF), and to improve the recognition and diagnostic ability for DTF. Methods: The clinicopathologic features of 14 cases DTF were analyzed, and the pathological features of the tumour were observed by using HE staining and immunohistochemistry (IHC). Results: 14 cases DTF were all female, and the median age was 31.5 years old (range 22-81 years old), 12 cases DTF were found in abdominal, and 2 in the right chest wall. In light microscope, the histologic features of DTF showed the proliferation of bundles of slide spindle cells across the collagenous fibres, cytological atypia was absent and pathological karyokinesis was rare. IHC showed a diffuse positive for Vimentin and β-catenin, partly for SMA, and negated for S-100, CD34, CD117 and Dog-1. Patients were followed up from 4 to 113 months after DTF resection, 1 case recurred. Conclusion: DTF is usually seen in young female, and needs to differentiate from many benign and malignant spindle cell tumors. It is of most importance for diagnosis and differential diagnosis of DTF that β-catenin is positive in the nuclear and cytoplasm. The main therapy is resection of DTF by operation, and the positive margins after operation may be indicate high recurrence risk.