Tailgut cysts (TGCs) are rare tumours which can undergo malignant transformation. The gold standard of treatment is complete surgical excision. Multidisciplinary assessment is advisable in order to apply adjuvant treatment. Postoperative radiotherapy should be given in case of compromised surgical margins or other factors such as high histological grade or perineural and lymphovascular invasion. Here, we present a case of a squamous cell carcinoma arising from a retrorectal TGC treated with surgery and postoperative radiotherapy and review the main indications and techniques of this therapy.