The Authors report a case of great desmoid tumor of the abdominal wall with intestinal adhesions and enterocutaneous fistula, in a patient with Gardner's syndrome, who underwent total colectomy with ileorectal anastomosis. The patient was treated at first with non steroidal antiinflammatory drugs, then with local chemotherapy. The Authors performed a second surgical procedure resecting the tumor and opening a temporary ileostomy. Despite of these treatment patient showed a local recurrence. Desmoid tumor is a neoplastic benign lesion arising from aponeurotic muscle tissue. Local infiltration and post-operative recurrence are very common. Several surgical and medical treatments are proposed, but they are not totally effective. Surgical treatment might be radical, with wide resection to reduce local recurrences. Radiotherapy and chemotherapy are second choice treatments.