Involvement of the kidneys in Henoch-Schönlein purpura (HSP) occurs in approximately 50% of patients with HSP, with varying severity. In general, disease outcome is favorable for adolescents. However, severe courses with vasculitis impairing multiple organ systems in addition to the kidney, including brain, heart, and intestine, may occur. This involvement, often manifesting more subtly, requires alertness for diagnosis and escalation of immunosuppressive therapy for treatment. We report a case of severe HSP nephritis with cardiac involvement in a young man. Cardiac involvement was noted initially on an electrocardiogram and visualized by using cardiac magnetic resonance imaging. HSP remission was induced with aggressive cytotoxic therapy, consisting of cyclophosphamide (750 mg/m(2) every 4 weeks) in addition to high-dose prednisolone. The case presentation is followed by a review of the literature for manifestations, treatments, and outcomes in patients with HSP complicated by cardiac involvement.