Congenital dislocation of the distal radioulnar joint has been rarely reported. In our case, a 20-year-old men was seen initially with pain in the right wrist two-years’ ago. Pain and swelling had increased with prolonged use of the hand two months before she was seen. No history of trauma could be elicited. Examination of her right hand and wrist revealed decreased range of motion of the wrist secondary to pain, without right wrist significantly swelling discomfort. Right lower ulnar joint area tenderness positive. The patient had not limited in pronation or supination of the forearm. Routine x-ray films of the right hand and wrist revealed dorsal dislocation of the distal radioulnar joint, positive ulnar variance is 0.9 mm. MRI of the right wrist revealed the triangular fibrocartilage complex (TFCC) is not injured or teared. However, wrist pain has continued despite conservative measures. Finally, the distal ulna were osteotomied, and made reduction and internal fixation with the plate. The patient have no pain and no dislocation of the distal radioulnar joint in the radiography at three month follow up.