Background: Squamous cell carcinoma (SCC) of the rectum is a rare malignancy, and retroperitoneal nodal metastasis (RNM) represent 1-2% of colorectal cancer metastasis (CRC), and radical retroperitoneal lymphadenectomy (RRL) in this setting remains controversial. However, there are no reports in the literature on how to treat SCC retroperitoneal lymph node metastasis. Case Presentation: The patient was a 47-years-old woman with SCC of the rectum. Concomitant chemoradiotherapy (CRT) followed by surgery was planned. After 8 weeks, CT of the abdomen for restaging showed a 6 cm para-aortic mass involving and obstructing the medium third of the left ureter. The patient was submitted to abdominoperineal rectal resection with left nephrectomy and en-bloc resection of para-aortic tumor (nodal bulking). Conclusion: After a thorough review of the literature regarding RNM for rectal SCC, we found no mention of how to treat RNM for rectal SCC. The purpose of this paper is to report a very rare case of RNM for rectal SCC and to discuss the surgical approach. Surgery seems to be the best option for local control and improvement in overall survival (OS).