A 14-year-old boy presented with acute paraparesis with sensory and sphincter disturbance. Imaging and neurophysiologic studies were diagnostic for transverse myelitis with acute motor axonal neuropathy. He was treated with both intravenous immunoglobulin and high-dose corticosteroids, and he made a slow and incomplete recovery. Acute motor axonal neuropathy is an unusual variant of Guillain-Barré syndrome. Concomitant transverse myelitis and acute motor axonal neuropathy were not previously reported in childhood, although there are reports of coexisting transverse myelitis and other variants of Guillain-Barré syndrome. Such dual lower motor neuron pathology may be associated with a poorer outcome, and indicates simultaneous central and peripheral immune-mediated injury. There may be a place for combined therapy with immunoglobulin and corticosteroids in patients with transverse myelitis and inflammatory neuropathy.