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Pediatric multiple sclerosis (MS), once considered a rare childhood illness, has been increasingly identified as an important childhood acquired neurologic disease requiring early recognition and intervention.We present a comprehensive review of the current terminology of acquired central nervous system demyelination in children, pertinent investigations, including magnetic resonance imaging and cerebrospinal fluid cerebrospinal fluid studies, and an approach to the differential diagnosis of pediatric onset MS. In addition, the recent studies exploring the epidemiology and pathobiology will be discussed. Finally, we present an algorithm for the treatment of episodes of demyelination along with chronic immunomodulatory therapeutic options in this patient population.Although some similarities exist to adult onset MS, MS onset during childhood and adolescence presents unique diagnostic challenges and requires specialized multidisciplinary care for optimal management. National and international collaborative studies are underway to aid in the understanding of the early and ongoing pathogenesis of MS.