Introduction: Synovial chondromatosis refers to a benign proliferative disease of the synovium. Reports in children are rare and reports involving the pediatric shoulder are even more rare. In this review of the literature and case report, we provide a summary of all published cases in children and a report of a case of shoulder synovial chondromatosis in an adolescent female athlete. Case Presentation: A 15-year-old female cheerleader presented to the clinic with gradual onset right shoulder pain with a severity of 5/10. On physician examination, the patient had tenderness over the proximal humerus, limited abduction to 160°, limited forward flexion, and a total arc of motion of <180°. Radiograph of the shoulder demonstrated multiple calcific intra-articular loose bodies consistent with synovial chondromatosis. Arthroscopic removal resulted in resolution of symptoms at the 36-week follow-up. Conclusions: Our literature search revealed only eight case reports on shoulder synovial chondromatosis in children. The location of the chondromatosis within the shoulder joint can be intra-articular, extra-articular, within the biceps sheath, or combined. In general, for chondomatoses confined to the glenohumeral joint space, open surgery is not required. Bony erosions can occur in the setting of chondromatosis though may not need to be addressed surgically. Although rare, this diagnosis should be considered when confronted with shoulder pain that is exacerbated during long athletic events with limited range of motion on physical examination in the adolescent athlete. Arthroscopic treatment in conjunction with physical therapy and early mobilization can lead to a successful outcome.