A review of the literature shows that the association of pyoderma gangrenosum and regional enteritis is rare. A case is reported of a 19-year-old woman who developed an ulcerative lesion of her left calf. The evolution and appearance of the ulcer was consistent with pyoderma gangrenosum. A biopsy of the ulcer showed a slit-like abscess in the deep dermis with giant cells surrounding it. Six months later regional enteritis was diagnosed. Prednisone administered orally gave the patient dramatic relief of the skin lesion. Following resection of the bowel for intestinal obstruction, the prednisone was discontinued, with subsequent relapse of the ulcer.