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Vasculitis is a vascular disorder characterized by inflammation of the blood vessels’ wall either as primary process or secondary to a systemic disorder. Though rare in paediatric age group, vasculitis may have a devastating and even fatal course. Vasculitides present with a wide range of clinical manifestation in childhood and they require a high index of suspicion for diagnosis. Vasculitis can be classified according to their clinical presentation, the size and location of the blood vessels affected, and histopathological patterns. Henoch- Schönlein purpura and Kawasaki disease are the two most common types of vasculitis occurring in children. Less common types include Takayasu arteritis, polyarteritis nodosa, Churg-Strauss syndrome, Wegener’s granulomatosis, microscopic polyangiitis, and others. Diagnosis of vasculitis is based on clinical, laboratory, and imaging features that should be collaborated to fulfil the established criteria for each form of the disease. Management depends on the stage of the disease (active disease, relapse, remission, or refractory disease) and should be tailored to each particular patient and each specific type of vasculitides. The aim of this article is to review the classification, clinical presentation, diagnosis, and management of different types of vasculitis in children. Keywords: Children, classification, clinical manifestations, epidemiology, management, paediatric, vasculitis.