Colitis cystica profunda is a rare intestinal lesion. Because of its clinical expression (rectorrhagia, mucorrhea and abdominal pain) and the way it appears to current imaging techniques this disease presents features which can be associated with colon neoplasm. Its diagnosis has to be confirmed histologically, and its etiology remains unclear. The following is a case report of colitis cystica profunda recurring 20 years after a first episode in a white woman, who had had an anterior resection of the sigmoid colon and upper rectum to deal with a colitis cystica profunda-induced stenosis of the sigmoid colon and at 41 underwent the transanal removal of a polypoid lesion. A review of 20 cases in the literature showed that colitis cystica profunda has a predilection for the male and generally affects the medial and lower rectum and the sigmoid colon. The literature also confirmed the association with ulcerative rectocolitis, Crohn's disease and rectal prolapse. The type of treatment varies from surgical, medical, and endoscopic to no treatment at all.