Tumors occurring in the retrorectal space are heterogeneous and uncommon. Multiple embryologie remnants derived from a variety of tissues are found in this space and this explains the heterogeneous pathology of cysts and tumors that arise in retrorectal space. Presacral cysts are rare in adults. Most of the lesions are benign. The cysts may be unilocular or multilocular. They are often asymptomatic. These tumors pose a diagnostic and therapeutic interest. Presacral epidermoid cysts are classified as a type of developmental cysts. We report the case of a 57-year-old woman who presented with a painless mass posterior of the anus. Imaging studies showed a multilocular cyst (65 × 100 × 60 mm) in the retrorectal space. The patient underwent surgery via a posterior approach. Histological study revealed a epidermoid cyst. A review of literature of this rare tumor is performed.

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