Peripheral nerve sheath tumours arising in the plexus or peripheral nerves can be treated by limb amputation. There are few reports of these tumours affecting peripheral nerves in the distal regions of the limbs. Here we describe a case of neurofibroma affecting the palmar branch of the ulnar nerve in an Irish setter. Surgical treatment in the region of the carpus by ulnar neurectomy resulted in resolution of chronic thoracic limb lameness. At 11 months following the surgery, clinical examination and MRI did not detect any evidence of recurrence. Neurectomy may be a feasible option for management of selected cases of distally located peripheral nerve sheath tumours.