The primary systemic vasculitides in childhood are quite rare except for Henoch-Schönlein purpura and Kawasaki disease. These are usually self-limited diseases. The etiopathogenesis for most of them is not clearly understood. However, it is usually thought that environmental triggers (mostly infectious) evoke an aggravated inflammatory response in susceptible individuals. The classification criteria for most of them were recently validated for children. Clinical manifestations are determined by the organs affected, reflecting the involved vessels on a background of constitutional symptoms. For treatment, the main data are adapted from adult studies, especially for rarer vasculitides in childhood. In this review, the recent classification criteria, current pathophysiologic pathways, and treatment protocols are summarized.