Intracranial epidermoid cysts are rare, comprising 0.2% to 1.8% of all primary intracranial expanding lesions, of which <5% occur within the fourth ventricle. Epidermoid cysts are frequently congenital, and patients often present in the fourth decade of life. These cysts produce symptoms as a result of mass effect on surrounding structures, most commonly the cerebellum and cranial nerves. Symptoms can include hearing impairment, trigeminal neuralgia (severe facial pain), facial tics, headaches, double vision, and facial palsy. However, no research has focused on the neuropsychological effects on a patient after surgical resection of these cysts. This case report presents the cognitive profile of a woman after resection of an epidermoid cyst in the fourth ventricle. The 49-year-old patient underwent neuropsychological assessment after removal of the cyst, completing a comprehensive set of cognitive tests of estimated premorbid intelligence, attention, memory, social cognition, language, visual perception, and executive functioning. Test results indicated executive dysfunction and reduced visuospatial memory in the acute stage after surgical removal of the epidermoid cyst. These findings suggest that cognitive deficits can occur after resection of space-occupying lesions in brain regions not typically associated with cognition. To our knowledge, this is the first report of the neuropsychological consequences of surgical removal of a congenital epidermoid cyst in the fourth ventricle. An understanding of the neuropsychological sequelae of this rare cerebral cyst will allow patients, families, and health professionals to better anticipate and manage postoperative difficulties.