Background: Squamous cell carcinoma of the rectum is a distinct entity. We report a rare case of squamous cell carcinoma of the middle and low rectum. Case presentation: The patient was a 60-year-old man who presented with a history of rectal Bleeding, constipation and pelvic painless. Rectoscopy and Colonoscopy revealed a polypoid tumour of the middle and low rectum. Biopsies of this mass revealed a poorly differentiated squamous cell carcinoma of the rectum. Pelvic Magnetic Resonance Imaging scan showed a polypoid, irregular and circumferential tumor in the middle and low rectum extending in length on 95mm. CT scan of the chest, abdomen and pelvis was negative for distal metastases. The patient received combined chemo-radiation. Overall treatment time was 45 days. After 6 weeks of the end of treatment, the revaluation clinical tests by pelvic magnetic resonnance imaging showed a poor response. After 3 months, Positron Emission Tomography with Fluorodeoxyglucose demonstrated a little hypermetabolic area with maximum standard up take value of 6,7 in the low rectum. Patient underwent a surgical resection. The postoperative histopathological findings were non specific and chronic inflammatory changes. At the time this report was written, the patient had 18 months of follow-up. No evidence of malignacy is found. Conclusion: Squamous cell carcinoma of the rectum is a distinct entity. Chemoradiation should be chosen as the first-line therapy for suqamous cell carcinoma of the rectum. However,a large study will be required to establish a safe and effective regimen.