Here, we report the first case of laparoscopy-assisted total colectomy for MEN2B-associated megacolon caused by intestinal ganglioneuromatosis.<h4>Presentation of case</h4>At age 13, he was evaluated for oral cavity masses, and was diagnosed with mucosal neuromas of his tongue, raising suspicion for MEN2B. This evidence concerns the gene RET and Aganglionic megacolon.