Primary synovial chondromatosis is an uncommon condition, and involvement of the glenohumeral joint is rare. Currently, the recommended treatment is arthroscope-assisted synovectomy and removal of the loose bodies. We report a case with the clinical findings, radiographic features, arthroscopic technique for treatment of this disease and the main histological appearance of the removed loose bodies. We believe this is an easy and safe method for management of this disorder and that the support of an experienced pathologist is necessary to avoid differential diagnostic problems with the uncommon malignant transformation.