PURPOSE OF THE STUDY: We report a case of complete unilateral absence of the radial artery in the forearm and reviewed the pertinent literature. CASE REPORT: An 18-year-old girl was admitted for multiple fractures after a car accident. She presented with a comminuted fracture of the left distal humerus, an open grade I fracture according to the Gustilo classification involving the right ulna and radius, a mediodiaphyseal fracture of the right femur and an open grade II fracture of the proximal and distal left tibia. After open reduction and internal fixation of the bones of the right forearm, she presented transient ischemia of her right hand, the radial pulse not being detectable at the end of surgery. An arteriography showed a complete absence of the right radial artery, which was thought to be caused by arterial thrombosis. Surgical exploration evidenced the complete absence of the radial artery. DISCUSSION: Absence of the radial artery is observed in radial preaxial hemimelia, in specific genetic and chromosomal disorders (Fanconi's anemia, Holt-Oram syndrome) and in association with other malformations. Unilateral absence of the radial artery has been described in association with other vascular abnormalities such as a larger anterior interosseous artery or the presence of a medial artery. Our case presented an isolated anatomical variation of the radial artery. This vascular anomaly was asymptomatic and discovered fortuitously. The incidence of this anatomic anomaly may be underestimated in the general population.