INTRODUCTION: Most of the primitive cystic neoplasms arising in the retrorectal presacral space are congenital. Presacral epidermoid cysts are classified as a type of developmental cysts, resulting from an error during the embryogenesis. They have a slowly-progressive growth that only lately can cause clinically remarkable symptoms. Most patients are middle-aged women. They are often asymptomatic. The discovery is almost always casual. It happens during controls like US, CT, MRI, gynecological visit. The surgical treatment must be effected for a correct diagnosis of nature of the lesion and to remove the mass. In fact, infective complications may occur and the increasing volume of the cyst can give clinical symptoms. Posterior approach is indicated for low or mid presacral space tumors; it is the most commonly followed route. The abdominal approach may be adequate for large developmental cysts. CASE REPORT: A case of an incidentally found large presacral epidermoid cyst in a young woman, which was excised through an abdominal approach is reported.