Tailgut cyst is a rare tumor originating from the embryonic remnant located in the retrorectal space. The diagnosis is usually incidental duse to the absence of symptoms. When present, they are nonspecific, such as abdominal pain, dysuria and tenesmus. Imaging tests are a great help in the diagnosis and surgical planning. The standard treatment is resection, which the surgeon must perform to avoid future complications, such as malignancy. We present a case of tailgut cyst in a young patient with prior pilonidal cyst excision, subsequently submitted to surgical resection, to share our experience with a rare case, with few reports in the literature.