Henoch-Schönlein purpura (HSP), the most common vasculitis that affects children, is an acute, small-vessel leukocytoclastic process. HSP is a systemic vasculitis involving vascular wall deposits of predominantly immunoglobulin (Ig) A within the small vessels of the gut, skin, joints, and kidneys, and in the mesangium of the renal glomeruli. The most frequent laboratory abnormalities are high erythrocyte sedimentation rates, microscopic hematuria, proteinuria, and elevated levels of IgA. HSP is known to cause neurological complications including seizure, chorea, encephalopathy, focal neurological signs, cortical blindness, as well as cranial and peripheral neuropathies and intracerebral hemorrhage. Ischemic infarction and strokes also occur in HSP. Pulse steroids have been shown to be effective, but sometimes plasmapheresis has been used to arrest disease progressio. Patients with severe nephritis, especially with the nephritic syndrome, have often been treated with corticosteroids and cyclophosphamide, cyclosporine, or azathioprine.