We present a rare case of a fourth ventricular epidermoid cyst, which recurred 15 years after the initial radical tumor resection surgery. The recurrence of the tumor extended into the cervical spine. The patient was reoperated and a near-total excision of both the cranial and spinal components of the epidermoid was performed. Our literature search did not reveal any case where there was a simultaneous presence of both the cranial and spinal epidermoid tumors. The possible cause of extension of the cranial epidermoid into the spinal compartment is analyzed.