A Riche (7)-Cannieu (2) anastomosis (ulnar-to-median anastomosis in the hand) in the setting of an ulnar or median nerve lesion can produce confusing clinical and electrodiagnostic findings. We report a patient with a deep branch ulnar neuropathy complicated by a Riche-Cannieu anastomosis. His clinical presentation led to an initial diagnosis of motor neuron disease. Extensive electrophysiologic studies clarified the extent of the Riche-Cannieu anastomosis and the ulnar neuropathy.