Synovial chondromatosis (SC) is a benign proliferative metaplasia of joint synovium into cartilaginous tissue. The relative scarcity of presentation in the wrist or hands contributes to the difficulties in accurately diagnosing and obtaining the necessary treatment for SC. We present a 43-year-old man with a 10-month history of stiffness and decreased range of motion of his right first metacarpophalangeal (MCP) joint. On examination, a firm mass was palpated surrounding his MCP joint. X-rays showed only a joint effusion. Subsequent magnetic resonance imaging (MRI) showed an effusion, high signal intensity joint distension, and small erosions. Surgical exploration and complete synovectomy with excision of loose bodies was performed. The pathological diagnosis, based on the clinical examination as well as MRI, was consistent with that of tenosynovial chondromatosis. This case illustrates surgical exploration and pathological investigations may be necessary for SC diagnosis. The literature was reviewed for greater understanding of SC's presentation, diagnosis, and treatment.