Objective: To investigate the preoperative evaluation, diagnosis and surgical approach of cholecystoduodenal fistula with gallstone ileus. Methods: The clinical data of a patient with cholecystoduodenal fistula and gallstone ileus were analyzed retrospectively, and the relevant literature was reviewed. Results: The patient was considered to have gallbladder stone and gallstone bowel obstruction by preoperative CT examinations. Intraoperative exploration found incarceration of a 40 cm stone at the ileocecal junction. Then, enterotomy and stone removal, duodenal fistula repair and cholecystectomy were successfully performed. The patient was discharged after postoperative recovery, and no related complications were observed up to the present time. Conclusion: Cholecystoduodenal fistula with gallstone ileus is a rare condition in clinical practice, early diagnosis and accurate assessment are critical, and appropriate surgical approach should be chosen according to the specific characteristics of individuals.