Purpose. To report a patient with primary squamous carcinoma of the re
ctum. Case report. A 40-year-old woman with hematochezia and change in
bower habits was studied. The main laboratory finding was a mild anem
ia. A barium enema and a proctoscopy revealed a rectal neoplasm at eig
ht cm from the anal verge. A transendoscopic biopsy demonstrated an sq
uamous rectal carcinoma. A transrectal ultrasound and CT scan of the a
bdomen revealed a big rectal mass with transmural affection and possib
le involvement of the lymph nodes. The carcinoembriogenic antigen (CEA
) was high (32 ng/mL). The patient underwent radiotherapy with 46 Gy,
and 5-fluorouracil as radiosensitizer. Three months later, a new CT sc
an showed significant reduction of the size of the mass, and the patie
nt underwent a very low anterior resection with double-stapled anastom
osis. The analysis of the specimen showed a squamous carcinoma of the
mid-rectum, invading through the wall without lymph node affection and
with proximal, distal, and radial margins free of tumor. The CEA retu
rned to normal after surgery (1.3 ng/mL). The patients is alive and wi
thout evidence of disease 18 months after the operation. Conclusion. P
rimary squamous carcinoma of the rectum is a rare disease, and surgery
seems to be a good option of treatment, with the possibility of sphin
cter preservation depending upon the location of the tumor.