The clinical and pathological features of 138 cases of fibroma of tendon sheath are presented. The tumor predominated between the 3rd and 5th decades; the youngest patient was 5 months of age, the oldest 70 years (median 31 years). The tumor was more common in males (75%) than in females; it affected chiefly the tendons and tendon sheaths of the fingers (49%), hands (21%), and wrist (12%). The main presenting symptom was an insidiously growing mass causing mild tenderness or pain in about one-third of the patients. The preoperative duration of symptoms ranged from 1 week to 6 years with a median of 5 months. The tumor was generally well circumscribed, and often lobulated. It measured between 1 and 2 cm in greatest diameter (median 1.8 cm), and in all but one of the cases was attached to tendon and/or tendon sheath. Microscopically, most were composed of dense fibrocollagenous tissue bearing dilated or slit-like vascular channels some of which resembled tenosynovial spaces. Although cellular variants focally mimicking fibrous histiocytoma and nodular fasciitis could be distinguished, there was always a transition from cellular toward the typical fibrocollagenous areas. Of the 54 patients with follow-up information (median follow-up period of 4.5 years) 40 or 74% were well with no evidence of recurrence in the follow-up period. In 10 patients, the tumor had recurred once and in 3 it had recurred twice. All of the recurrences were in the fingers or the hands. The overall recurrence rate was 24% which is comparable to that of giant cell tumor of tendon sheath. One patient died of unrelated cause. Local excision appears to be the treatment of choice for these tumors, including the lesions that recur.