Lymphangiomatosis of the colon is an extremely rare disease. We report here a case of a 40 year old woman with colonic lymphangiomatosis who was treated by surgical resection. Computed tomography of the abdomen and pelvis showed a multiple cystic mass encasing the descending colon. However, although colonoscopic examination did not reveal any abnormalities, a laparotomy revealed cystic masses on the entire colon; therefore a near total colectomy was performed. There was no sign of recurrence after 14 months of follow up. Histology revealed lymphangiomatosis of the colon. Colonic lymphangiomatosis is an uncommon disorder, and the occurrence of a case of lymphangiomatosis that cannot be detected by colonoscopy is extremely rare. Therefore, this report should be helpful for the management of patients with lymphangiomatosis.