Malignant duodenocolic fistula is a rare complication of gastrointestinal malignancy. We present herein the case of a 34-year-old female in whom a large duodenocolic fistula was caused by advanced transverse colonic carcinoma. Right hemicolectomy combined with pancreaticoduodenectomy enabled en bloc resection of the tumor, and the patient has been free of disease for 1 year and 8 months postoperatively. A review of the international literature, including 33 cases reported in Japan, indicates that if the disease is curable, the treatment of choice is right hemicolectomy with pancreaticoduodenectomy, whereas if it is not curable but locally resectable, the best palliation appears to be right hemicolectomy with partial duodenectomy to include the fistulous tract. Dehiscence of the duodenal wound closure associated with partial duodenectomy can be prevented by using the mucosal or serosal patch techniques with intestinal loops. These therapeutic principles are also applicable for colonic carcinoma which massively involves the duodenum without fistula formation.