A 31-year-old white woman with biopsy verified cutaneous polyarteritis nodosa diagnosed in 1981, developed 6 years later recurrent abdominal pains, rectal bleeding and weight loss. Barium enema demonstrated typical changes of Crohn's disease with fistula in the terminal ileum. Her resected ileum showed granulomatous transmural ileitis without vasculitis. Only corticosteroids and cyclophosphamide controlled the cutaneous and the gastrointestinal symptoms until she was treated with sulfasalazine. The association between cutaneous polyarteritis nodosa and Crohn's disease is discussed and the treatment of earlier reported cases is reviewed.