Acute transverse myelitis is a clinical syndrome affecting the spinal cord, which is characterized by acute onset of motor, sensory, and autonomic dysfunction. Approximately 20% of cases of acute transverse myelitis occur in children. This review summarizes the current published literature on acute transverse myelitis, including epidemiology, diagnostic criteria, pathogenesis, clinical presentation, clinical evaluation, and differential diagnosis. The article also summarizes the neuroimaging features, acute and chronic complications, treatments, and prognosis of acute transverse myelitis in the pediatric population. The initial evaluation centers on differentiation from other causes of myelopathy, and cases are further divided into idiopathic or disease-associated acute transverse myelitis. Correct diagnosis is important for treatment and prognosis. Treatment begins with intensive surveillance for acute life-threatening respiratory or autonomic complications. Immunomodulating therapy is recommended for noninfectious causes, using high-dose intravenous corticosteroids or plasma exchange. Other therapeutic options are also discussed. Prognosis depends on a number of factors, and evidence suggests that the majority of children have a good outcome. A small percentage of children diagnosed with acute transverse myelitis later are diagnosed with other demyelinating diseases, especially neuromyelitis optica, or multiple sclerosis. The most common long-term complications of acute transverse myelitis are urinary, motor, or sensory dysfunction.