Granuloma annulare (GA) is a self-limited benign inflammatory dermatosis of unknown origin characterized by the development of papules arranged in an annular, circinate pattern, mainly involving the extensor surfaces of lower and upper extremities. The diagnosis depends on clinical and histological features. There are four main variants of GA: localized GA (LGA), subcutaneous GA (SGA), generalized GA (GGA) and perforating GA (PGA) and 2 uncommon subtypes: papular umbilicated GA and linear or segmental GA. Children most commonly present with the localized and subcutaneous forms. The aim of this review was to provide an update on the etiology, diagnosis and management of GA focusing on the pediatric age.