Background: Isolated neuropathy of the dorsal cutaneous branch of the ulnar nerve (DCBUN) is rare and most cases are secondary to trauma, often iatrogenic. The topography of sensory abnormalities and abnormal electrodiagnostic (EDX) findings are crucial in confirming DCBUN neuropathy.Materials and methods: This is a retrospective study of patients with isolated involvement of the DCBUN from among patients referred for EDX studies for upper extremity symptoms. All patients underwent a focused neurological examination followed by EDX studies. Ultrasound (US) studies were performed in two patients.Results: Of the 14 patients with DCBUN neuropathy, decreased pinprick sensation in the distribution of the DCBUN was noted in 11 (78%) patients. DCBUN sensory nerve action potential (SNAP) was not recordable in 13 (92%) patients. In one patient who had a recordable SNAP, the latency was prolonged, and the amplitude was decreased. Four (28%) patients had incidental EDX abnormalities suggestive of entrapment of the median nerve at the carpal tunnel. The most common cause of DCBUN neuropathy was trauma in 13 (92%) patients, of which eight were iatrogenic. No specific etiology was detected in one patient (7%). Of the two patients who underwent US studies, one had increased cross-sectional area (CSA) at the wrist with prominent fascicles and hyperechoic scar tissue, while the CSA was normal in the other patient.Conclusions: Although rare, DCBUN neuropathy can be readily confirmed by typical clinical features and EDX findings. Surgeons should be aware of the anatomy and clinical features of DCBUN neuropathy and avoid injuring the nerve during surgical procedures at the wrist and forearm.