When children present with acute myelopathy manifested by sensory, motor, or bowel and bladder symptoms, MRI of the neuraxis with contrast agent is the most important imaging study to obtain. Although occasionally normal, MRI often demonstrates signal abnormality within the spinal cord. Classically, longitudinally extensive transverse myelitis (≥3 vertebral bodies in length) has been described with neuromyelitis optica (NMO), but alternative diagnoses should be considered. This pictorial essay reviews the differential diagnoses that may present with longitudinally extensive spinal cord signal abnormalities. Multiple inflammatory, infectious, vascular, metabolic and neurodegenerative etiologies can present with a myelopathy. Thus, radiologists can assist in the diagnosis by familiarizing themselves with the spectrum of diseases in childhood that result in longitudinally extensive signal abnormalities in the absence of trauma.