In 1941, Jaffe and coworkers studied a lesion with histologic appearance of fibrous stroma, pigmented deposition and histiocytic infiltration as well as giant cell, for which they named pigmented villonodular synovitis, bursitis and tenosynovitis. Thereafter many authors have discussed etiology, clinical and radiological features, pathology and treatment regarding the disease. Almost all, this disease is monoarticular and knee joint is most common site. We experienced a case of localized pigmented villonodular synovitis involving an shoulder joint which was treated by local excision and obtained good result.