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Arthritis of the distal radioulnar joint is multifactorial; the most common causes are trauma, congenital anomalies, degenerative, and inflammatory diseases. Women are more commonly affected by congenital anomalies like Madelung deformity as well as degenerative and inflammatory arthritis. Trauma equally affects men and women of all ages. The functions of the forearm include rotation and stability and the capability of transferring loads or bearing weight; all are dependant on the normal anatomic relationships about the radioulnar joints (proximal and distal), the interosseous membrane, and the shape of the forearm bones. Pathologies affecting any of these structures result in pain, decreased strength, limited range of motion, and loss of overall forearm function. The type of arthritis involving the individual will determine the scope of the treatment. Impending arthritis from trauma can be prevented by early interventions in the form of corrective osteotomy for malunited distal radius and distal ulna fractures, ligament reconstruction when the triangular fibrocartilage has been ruptured or detached, and ulnar shortening to treat early osteoarthritis of the distal radioulnar joint. Ulnar shortening changes the contact between the distal radius and ulna, extending in this way the life of the joint. Finally, when the joint is beyond repair, it is necessary to replace it with a stable prosthesis that restores the function of the distal radioulnar joint, allowing weight-lifting and pain-free motion.