OBJECTIVE To present a pathology not previously informed in national literature. BACKGROUND Colitis cystica profunda (CCP) is a rare benign entity and with an unknown etiology. There are 148 cases documented in world literature at the present time. Histologically it is defined as the obliteration of lamina propria by fibroblasts and the presence of submucous cysts. It comes in localized, segmental or diffuse forms and associated to other entities as solitary rectal ulcer, rectal prolapse, alterations in the pelvic floor, etc. Treatment can be medical or surgical according to severity of its manifestations. METHODS A case of CCP is revised as well as the treatment effectuated. RESULTS A case of patient with the diagnosis of CCP of localized type is presented, which principal symptom was transanal mucous secretion; on physical exploration a sessile mucous polypoid formation at 8 cm from anal verge was found and was treated with surgical resection with a satisfactory outcome. CONCLUSIONS In a patient with alterations in pelvic floor or puborectal bundle contraction and sigmoidorectal intussusception CCP must be suspected. Treatment can be medical with a high percentage of failure being optimal treatment the surgical one.