A 16-y-old boy presented with complaints of acute abdomen. Ultrasonography showed an enlarged and tender appendix suggestive of acute appendicitis. The patient underwent surgery and during surgery the appendix was found with a red vasculitis-like distal end, not typical for acute appendicitis. Further per operative examination of the intestines revealed a circular, vasculitis-like red band in terminal ileum. Four days after surgery, the patient developed skin purpura and complained of joint pain classical of Henoch-Schönlein disease. Pathological evaluation of the appendix and a skin biopsy confirmed the diagnosis and the patient was managed accordingly.