Epidermoid cysts originating in the paramedian basal cisterns of the posterior fossa are congenital lesions that grow to a large size through slow accumulation of desquamated epithelium. These lesions grow between and ultimately displace cranial nerves, vascular structures, and the brain stem, causing a long course of progressive neurological deficits. The onset of symptoms usually occurs during the fourth decade of life. Epidermoid cysts are easily diagnosed with computerized tomography scans, which characteristically show a low-density extra-axial pattern. The primary surgical objective is to decompress the mass by evacuating the cyst contents and removing nonadherent portions of the tumor capsule; portions of the capsule adherent to vital structures should be left undisturbed. Aseptic meningitis is the most common cause of postoperative morbidity, and its incidence may be minimized by intraoperative irrigation with steroids followed by systemic therapy with dexamethasone. Symptomatic recurrences that occur many years after surgery should be managed with conservative reoperation.