A 66-year-old patient was admitted to a hospital because of suspicion of secondary pneumonia after acute tonsillitis. By the findings of arthralgia, abdominal pain, nephropathy with urinary protein and uric blood, palpable purpura on the lower extremities, and a histopathology of skin biopsy, he was diagnosed with IgA vasculitis (Henoch-Schönlein purpura). Despite intravenous steroid pulse therapy, the patient had alveolar hemorrhage, and mechanical ventilation was required resulting from progressive respiratory failure. The vasculitis had developed in both lungs, kidney, skin, digestive tract, and brain. The patient had prolonged impaired consciousness and convulsion because of cerebral vasculitis. Two cycles of plasma exchange were performed, and systemic vasculitis was improved. The patient remains disease-free for two years after discontinuation of steroid therapy. We report here a first case of fulminant IgA vasculitis accompanied by alveolar hemorrhage and cerebral vasculitis, which were successfully treated by plasmapheresis.