Primary articular synovial chondromatosis is a benign, self-limiting neoplastic process in which hyaline cartilage nodules form in the synovial tissue. The disease most frequently affects the knee in men, followed by the elbow. The basic feature of this disease is a metaplastic maturation of the mesenchymal cells in the synovial membrane of a joint into cartilage. These cells mature into chondroblasts and form small nodules of cartilage in the synovial membrane. These nodules subsequently enlarge and detach to lie within the joint space. They become free within the joint as multiple small cartilaginous loose bodies nourished by the synovial fluid. The chondrocytes in the loose bodies continue to multiply, and the loose bodies grow in diameter. Calcification appears in the central zone of the loose bodies, and in some cases, enchondral ossification takes place. The operative therapy depends on the stage of the disease: synovectomy with removal of chondral fragments if active intrasynovial disease is present, and removal of the multiple chondral bodies alone in cases of late inactive disease with no synovial abnormalities. Malignant transformation is unusual and can be difficult to distinguish from benign disease.