Radial longitudinal deficiency is a spectrum of upper extremity dysplasia and hypoplasia affecting the proximal arm and the radial aspect of the forearm, wrist, and hand. Often, the hand surgeon is the first to evaluate a patient with radial longitudinal deficiency and thus must be aware of its common associated syndromes. Specific evaluation, including clinical examination and laboratory testing, is necessary. At this time, there are many surgical approaches that can be used for treatment of radial longitudinal deficiency. The procedures should be specifically tailored to the patient and family to improve overall function and clinical outcome.