Essex-Lopresti injuries are rare and present a clinical challenge. Incomplete diagnosis and treatment can lead to long-term instability, pain and functional impairment. We report on a clinical case of proximal radioulnar joint (PRUJ) and distal radioulnar joint (DRUJ) dislocation with unusual distal radial displacement and associated radial head shear fracture. The case was managed with closed reduction of the PRUJ and DRUJ followed by open reduction and fixation of the radial head. A high index of suspicion with thorough examination of the elbow, forearm and wrist and comprehensive imaging was important in reaching a complete diagnosis for appropriate treatment. Anatomical reduction of the PRUJ and DRUJ is essential to achieve optimal functional outcomes. Six months following the injury the patient made a satisfactory recovery with full range of movement; however, she continued to have mild to moderate general and moderate work-related disability.