Background: Pigmented villonodular synovitis (PVNS) occurs in three forms: giant cell tumors of tendon sheaths or bursae, diffuse PVNS of joints, and localized PVNS of joints. Both the diffuse form and the localized form of PVNS of joints are rare disease entities that most often affect the knee joint. The over-all annual incidence rate is 1.8 patients per million in the general population. Six cases of intra-articular localized PVNS were reviewed in this study. Methods: The records of all six patients who had received surgical treatment and pathology-proven localized PVNS of joints between June 1988 and February 1999 were reviewed. The material review included clinical notes, operative records, pathological reports, preoperative and postoperative imaging studies, and histological reports. All patients were called for a follow-up evaluation, and all of them were seen and examined. Results: There were two men and four women. The average age was 37 years (range, 19 to69 years). PVNS was located in the knee joints and managed with marginal excision by either open arthrotomy or arthroscopic operation in all cases. There was no evidence of recurrence at the follow-up evaluation (range, 12 to 126 months: average, 56.3 months). The average duration from onset of symptoms to presentation was 10.7 months-(range, 3 to 36 months). Discomfort was the most common complaint in the clinic (6/6). Conclusions: Preoperative diagnosis is difficult without the use of MR imaging. Painless hemarthrosis is not as common as that reported in the literature, and the ages of these cases are younger than those of patients with diffuse PVNS. The symptoms are more acute in cases of localized PVNS of joints. Marginal excision is the appropriate treatment for patients with localized PVNS.