Henoch-Schönlein purpura (HSP) is a systemic small vessel vasculitis mainly affecting children. We report a case of a 49-year-old woman with severe gastrointestinal and renal involvement of HSP. Endoscopy revealed more characteristic findings in the terminal ileum than in the gastric antrum. Histological examinations of the biopsy samples from the ileum, antrum, skin and kidney confirmed the diagnosis of HSP. Parenteral corticosteroid therapy led to a rapid improvement of the gastrointestinal symptoms, but because of the excessive proteinuria intravenous cyclophosphamide therapy had to be introduced.