Introduction: Perirectal epidermoid cyst is a rare mass arising from the ectodermal germ cell layer of the hind gut, which is most often found in middle-aged women. It is difficult for us to make an accurate diagnosis and distinguish from other developmental cysts. Case Presentation: Here, we report a 19-year-old girl who had suffered from sacrococcygeal falls and burns on the left buttocks in the growth experience. There was a scar on the left buttocks, and a soft mass with sacral deformity was seen in the sacrococcygeal region. According to the results of the sacrococcygeal CT and pelvic MRI, the preoperative diagnosis included presacral epidermoid cyst, anal sinus and sacrococcygeal scoliosis. Finally, the cyst was completely resected posteriorly through sacrococcygeal approach. Pathology was verified as an epidermoid cyst. The patient stayed recurrence-free after six months of follow up.Conclusion: Perirectal epidermoid cyst is a rare mass that should be considered in the differential diagnosis of pararectal cysts. It is of significant for the approach of surgery and prognosis.