We reported a case of idiopathic megacolon with extremely elongated sigmoid colon. A 30-year-old man was admitted to our hospital because of abdominal pain, abdominal fullness, and constipation. A plain abdominal x-ray showed extremely dilated colon, measuring 15cm in diameter. Gastrographin enema study showed the dilated rectum and sigmoid colon with elongated sigmoid colon. Conservative therapies were ineffective. At laparotomy, the rectum and sigmoid colon measured 90 cm in length dilated and the descending colon incompletely fixed to the retroperitoneum. Resection of the dilated rectum and sigmoid colon was performed with end-to-end anastomosis. Since histological examination revealed that the ganglion cells of the rectum were normal, the patient was diagrosed as having an idiopathic megacolon. There is no recurrence of megacolon after the operation.