Perianal abscess (PA) and fistula-in-ano (FIA) are common entities in infancy. Although several hypotheses have been suggested, the pathogenesis of PA/FIA remains elusive. The natural course of these diseases in infancy is self-limiting in the majority of cases whereas older children show similarities to PA/FIA in adults. It is important to rule out rare differential diagnoses of PA/FIA such as inflammatory bowel disease (IBD), surgical complications after colorectal surgery, and immunodeficiencies. Treatment remains empiric, comprises conservative, as well as surgical approaches, and is dependent on the age of the patient. This review summarizes anatomical aspects, current evidence on disease pathogenesis, clinical presentation, and management of pediatric patients with PA and FIA.