Neuroleptic malignant syndrome (NMS) is a severe iatrogenic complication of treatment with antipsychotic medication. The aim of this review is to provide the clinical characteristics and treatments of children and adolescents with NMS. Searches were conducted in Medline, Korean studies Information Service System (KISS), and Research Information Service System (RISS). Sixteen case reports and two review articles were selected in Medline, and two Korean cases reported in department of emergency medicine and pediatrics were selected from RISS. Heterogeneous and atypical presentations of NMS were observed in children and adolescents. Some noticeable differences were observed between adult patients and child patients with NMS. In addition, symptom presentations related to atypical antipsychotic agents differed from those of typical ones. In treatment, bromocriptine and benzodiazepine were recommended for management of symptoms. In particular, electroconvulsive therapy (ECT) was a useful treatment option. For prevention and early detection of NMS in children and adolescents, evaluation of risk factors and understanding of diagnostic features of NMS are very important.