A tailgut cyst is a type of benign congenital disease that mainly develops in the retro-rectal space. However, malignant transition can occur in some cases of tailgut cysts. Early and precise diagnosis, and proper treatment, are vital for patients with tailgut cysts with malignant transformation. In this review, we aim to summarize the similarities and differences in the diagnosis and treatment methods among the 3 most frequently reported types of tailgut cysts with malignant transformation. In our study, PubMed and Web of Science databases were used to search for the studies and the key words were "tailgut cysts" and "malignancy". We found 176 articles and selected 75 articles in our survey, with 9 reviews, 35 case reports, and 31 case reports and reviews.