Radial longitudinal deficiency (RLD) is a congenital disorder characterized by hypoplasia of both the radius and soft tissue structures on the preaxial side of the forearm. Clinical presentation can vary within a spectrum of severity ranging from mild cosmetic deformity to complete absence of the radius and thumb. Despite over 250 years of investigation, current surgical treatment of this disorder is still inadequate in restoring "normal" function and appearance within the wrist. Shortcomings in surgical management have made a standardized treatment algorithm for RLD controversial; however centralization and radialization remain the mainstay of surgical treatment. This chapter will attempt to provide an overview of the deformity and present a review of treatment options.