Knowledge of anorexia nervosa (AN) in childhood is scarce. This review gives a state-of-the-art overview on the definition, classification, epidemiology and etiology of this serious disorder. The typical features of childhood AN in comparison to adolescent AN and avoidant restrictive eating disorder (ARFID) are described. Other important issues discussed in this article are somatic and psychiatric comorbidity, differential diagnoses and medical and psychological assessment of young patients with AN. Special problems in the medical and psychological treatment of AN in children are listed, although very few studies have investigated age-specific treatment strategies. The physical and mental outcomes of childhood AN appear to be worse than those of adolescent AN, although the causes for these outcomes are unclear. There is an urgent need for ongoing intensive research to reduce the consequences of this debilitating disorder of childhood and to help patients recover.