A case of giant epidermoid which was mainly located in the right temporal lobe was presented. The intracranial epidermoid is commonly located in the CSF space, such as the cerebello-pontine (C-P) angle, parasellar region, middle cranial fossa and ventricles. The intramedullary epidermoid is extremely rare. We report the case of a 51-year-old female suffering from generalized convulsions. Having fallen into a semicoma due to the convulsions, she was referred to our department on August 5, 1992. Neurological examination revealed slight dementia but no other deficits. The tumor was mainly located in the temporal lobe but communicated with the CSF space of C-P angle through a narrow channel shown on MRI. She underwent an operation for tumor removal and left the hospital with no neurological deficits. Several cases of intracranial intramedullary epidermoid have been reported in the literature, but none of them proved to be completely intramedullary. On operation or on CT and MRI, all of them were shown to communicate with the CSF space. The possible occurrence of an epidermoid from medullary substance was discussed.