Introduction: Primary synovial osteo chondromatosis (PSOC) is chondroid metaplasia with multi¬nodular proliferation of the synovial lining of a diarthrodial joint, bursa, or tendon sheath. It usually occurs in third-fifth decade and shoulder joint involvement is infrequent. It is very rare in children and primary extraarticular PSOC of the shoulder has been reported very rarely in children.

Case Report: We present a case of primary PSOC of the long head of biceps in 8year child. It presented as painful swelling in proximal arm. The pain radiograph was showing multiple calcified loose bodies on anteromedial aspect of humerus. MRI scan showed fluid filled cysts with calcified wall. The lesion was excised through deltopectoral approach. He had complete resolution of symptoms without recurrence at 1 year. The diagnosis was confirmed on histopathological examination.

Conclusion: We want to emphasize that one should keep a differential diagnosis of this rare condition in patients presenting with cystic swelling with calcified wall. We further emphasize the need to follow these patients to detect recurrence or malignant transformation.

Keywords: PSOC, Children, Biceps Tendon.

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