A 32-year-old male patient presenting with huge varices involving the sigmoid colon and the entire rectum down to the dentate line is described. There was no familial history of gastrointestinal bleeding. No particular aetiology was identified. Marked anaemia due to recurrent bleeding required resection of the sigmoid colon and rectum. Restoration of the intestinal continuity was performed by a coloanal anastomosis. The patient did well and bleeding has not recurred. To our knowledge, no similar case of such varices requiring extensive rectal surgery to control bleeding has been described in the literature.