Acute transverse myelitis (ATM) is a potentially devastating immune-mediated disorder of the spinal cord that affects all ages. Typical ATM is a monofocal and monophasic inflammatory disorder targeting primarily the spinal cord, resulting in motor, sensory, and autonomic dysfunction. ATM presents with sudden onset of rapidly progressive weakness that most often involves the lower extremities but may include the arms as well. Abnormal findings were reported in four out of six children with ATM who underwent spinal cord MRI. A case series of ATM in childhood reported functional outcomes in a group of 33 individuals using the FIM and WeeFIM tools for measuring performance of daily skills at an average follow-up of eight years following the onset of disease. The initial phase of Guillain-Barré syndrome (GBS) may be confused with ATM because of the similar presentation of muscle weakness, loss of reflexes and meningeal signs.