We report herein the unusual case of a 55-year-old man who presented with primary Crohn's disease of the appendix with fistulae. In this patient there was no history of previous appendectomy. Although fistula formation around the orifice of the appendix after appendectomy for primary Crohn's disease of the appendix has been observed in a number of long-term follow-up studies, preoperative fistula formation is extremely rare.