We describe a case of primary Hodgkin disease of the terminal ileum in a 38-year-old man with Crohn disease of 24 years' duration. The infiltrate was located in an ulcerated fistula involving the terminal ileum and urinary bladder. Reed-Sternberg cells and their variants were characteristically positive for CD15, fascin, and CD30 and showed focal positivity for CD20. Epstein-Barr virus messenger RNA was also detected in the neoplastic cells. Staging revealed no evidence of other lymph node or organ involvement. Although rare, primary gastrointestinal Hodgkin disease arising in the setting of Crohn disease may have a stronger association with Epstein-Barr virus infection than conventional Hodgkin disease.