Synovial chondromatosis is a rare, usually benign disorder affecting the population predominantly in the third and fourth decades of life and mainly involving the large weightbearing joints of the lower limb-the knees and the hip. In this report, we highlight an unusual pediatric clinical presentation of synovial osteochondromatosis involving the subtalar joint and discuss its surgical management; we also provide a comprehensive up-to-date literature review of the disorder. This patient was successfully treated with en masse surgical excision. He has been doing well, with complete pain relief and improved range of motion at 1-year follow-up. An exceptional involvement of the subtalar joint and an unusual presentation in the pediatric age group makes this case unique.