Carpal tunnel syndrome (CTS) is a frequent neuropathic condition that can result in numbness, pain, and tingling sensations in the fingers and hand. It often affects individuals who have specific risk factors, such as injuries, pregnancy or certain medical disorders. In this case report, we describe a patient presenting with symptoms of carpal tunnel syndrome (CTS), along with radial longitudinal deficiency, which represents a spectrum of upper limb dysplasias and hypoplasias. The patient exhibited unilateral thumb and radial artery hypoplasia, with the ulnar artery being the exclusive source of blood supply to the hand. Initially, the patient reported mild CTS symptoms that gradually worsened, impairing her daily activities. An ultrasound confirmed the diagnosis, and surgical release of the carpal tunnel was performed. Following surgery, the patient reported significant improvement in her symptoms, with her hand and finger strength and sensation returning to baseline. The aim of this case report is to enhance the knowledge and skills of orthopedic practitioners by presenting a detailed account of the diagnosis and management of carpal tunnel syndrome in a patient with oligodactyly and an unique anatomical variation of hand vascularity. We discuss the topic of radial longitudinal deficiency and typical anatomical variations in human hand vascularity.