Background: The presacral space, which contains different types of embryonic tissue, is a potential site for several tumors including epidermoid cyst. Presacral cysts are divided into two major groups: teratomas and developmental cysts. Presacral developmental cysts are rare congenital injuries with significant manifestations in the adult. Our objective was to report a case of a giant epidermoid presacral and retrorectal cyst.

Clinical Case: We present the case of a 28-year-old female whose clinical feature was the presence of a perianal mass and difficult micturition of 8 years. Rectosigmoidoscopy and videocolonoscopy were normal. Simple and contrast abdominal and pelvic tomography (CT) were ordered as well as nuclear magnetic resonance (NMR) imaging to determine extension and location of the tumor. These studies demonstrated three tumors in the presacral space. Surgery using a combined abdominal and perianal approach was done. Pathological report was epidermoid cysts. The patient had a favorable evolution with no reports of fecal incontinence.

Conclusions: The accepted definition describing epidermoid cyst is squamous stratified epithelium with keratohyaline grains, but with no other skin structures. Developmental cysts are slow growing due to their unique location, despite being congenital. Diagnosis is confirmed by ultrasound, CT, and NMR. Biopsy is contraindicated. Three described approaches are abdominal, combined or abdominoposterior, and transsacral, each with its specific indications.

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