The intra-articular space is a relatively rare site of occurrence of neoplastic diseases. The 2 distinct groups of clinicopathologic entities that exhibit an almost exclusive tropism for the joints are represented by synovial chondromatosis and tenosynovial giant cell tumors (TGCT). Synovial chondromatosis is a locally aggressive chondrogenic neoplasm that very rarely can show malignant behavior. TGCT occur in 2 main variants, the localized variant and the more locally aggressive diffuse type. Malignant TCGT is exceedingly rare and is characterized by significant rates of both local recurrence and metastatic spread.