A 61 year-old female presented with abdominal pain, rectal bleeding, mucus discharge, tenesmus and constipation. Rectal examination and proctoscopy demonstrated rectal stenosis at 5 cm from the anal verge. Transrectal ultrasonography detected a capsulated lesion as a mesenchymal rectal tumor. Computed tomography and endorectal magnetic resonance detected a mesenchymal lesion in the lower-middle rectal thirds. Serum TPA, GICA, SCC and CYFRA were pathological. At surgery the tumour was fixed to the levator ani muscle with rectal folding. Frozen sections of the levator ani muscle biopsies revealed cloacogenic tumour. Abdominoperineal resection was performed. The rectal lesion was cloacogenic carcinoma at 9 cm from the dentate line (pT4 pN0; Ki67 35%; CD31 181 vessels/mm2). Adjuvant radio-chemotherapy was performed. The patient is alive and disease free at 19 months. Extra-anal cloacogenic tumours are an unusual finding. Perhaps cloacal cells were originally present in the rectal wall, but secondary rectal involvement by cloacal remnant from the levator ani muscle cannot be excluded.