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Primary synovial chondromatosis is a benign condition and consists of cartilaginous metaplasia of the synovium, which leads to the production of intra-articular and periarticular osteocartilaginous bodies. The entity usually involves the synovium of large joints (knee, hip, elbow, and shoulder). It has also been reported in the hand, especially involving interphalangeal or metacarpal joints of digits. The most common symptoms are pain, swelling, loss of range of motion and muscle atrophy. Because of its low prevalence and non-specific symptoms, synovial chondromatosis can present a diagnostic challenge for the hand surgeon and may lead to a delay in treatment. To date, only three cases involving distal interphalangeal joint have been reported. This article describes a rare case of primary articular synovial chondromatosis in the distal interphalangeal joint of a 65-year-old female, giving a deep insight into this …