The surgical management of the Lynch syndrome patient with colorectal cancer needs to be individualized. Because of the increased incidence of synchronous and metachronous colorectal neoplasms, most favor an extended resection at the time of diagnosis of colorectal cancer. Age of diagnosis, stage of the tumor, co-morbidities, surgical expertise, surgical morbidity, and patient wishes should be taken into account when considering a surgical procedure. There are no prospective randomized trials or retrospective trials suggesting that patients undergoing an extended procedure have a survival advantage compared to those undergoing segmental resection. In retrospective studies it has been demonstrated that patients undergoing extended procedures will develop less metachronous colorectal neoplasms and will undergo less subsequent surgical procedures related to colorectal cancer. In females abdominal hysterectomy and bilateral salpingoophorectomy should be considered at the time of surgery for colorectal cancer.