Krabbe disease is an autosomal recessive disease that is associated with central and peripheral demyelination, and nerve conduction studies typically show sensory motor demyelinating polyneuropathy. We studied a patient with adult-onset Krabbe disease presenting motor dominant peripheral neuropathy. The patient was admitted at his age of 32, with 4-year history of slowly progressive gait disturbance. He presented upper and lower pyramidal tract sign. Brain fluid-attenuated inversion recovery MRI demonstrated bilateral high signal intensity areas in precentral gyrus, posterior limb of internal capsule and optic radiation. Laboratory analysis documented low enzyme activity of galactocerebrosidase and vitamin B12 deficiency. We diagnosed Krabbe disease with vitamin B12 deficiency. Nerve conduction studies showed motor dominant, mixed axonal-demyelinating neuropathy; prolonged F-wave latency in the median nerve, low amplitude compound muscle action potential (CMAP) in the ulnar nerve, slowing of motor conduction velocity, prolonged motor distal latency and low amplitude CMAP in the tibial nerve, and intact sensory nerve conduction studies. The pattern of peripheral neuropathy in adult-onset Krabbe disease could be variable.