Synovial osteochondromatosis of the distal radioulnar joint is a rare entity with only 14 cases reported in the literature. Malignant transformation of synovial osteochondromatosis is the most worrisome complication of the disease. It has been described in joints such as the hip and knee but never for the distal radioulnar joint. We report a case of synovial osteochondromatosis of the distal radioulnar joint which presented with radiographic features which were worrisome for malignant transformation and required a comprehensive preoperative workup. Discussed are the preoperative management, surgical treatment, and a literature review of this rare disease.