A nine-week-old saluki puppy was presented to Tufts University School of Veterinary Medicine for progressive, generalized weakness and bilateral forelimb deformities. Examination suggested a diffuse neuromuscular lesion. Cerebrospinal fluid (CSF) analysis showed normal nucleated cell count and protein level; however, many macrophages had vacuolated cytoplasm. Electromyography (EMG) recordings suggested denervation in paraspinal and appendicular muscles. Tibial motor nerve conduction velocity was normal, but direct evoked muscle potential had reduced amplitude. Histopathology revealed diffuse, symmetrical, degenerative motor neuronopathy of the ventral horn of the spinal cord with associated lesions in nerves and muscles. Histopathology was consistent with an abiotrophy that was likely inherited.