The authors describe a case of intracranial epidermoid cyst with fourth ventricle involvement. A 43-year-old woman presented with progressive aggravating headache and cerebellar syndrome due to a large tumor in the fourth ventricle, that extended into the left side of foramen of Luschka. The histopathological workup following gross total removal of tumor had revealed the typical features of an epidermoid cyst. Four months after surgery, the cerebellar sings recovered to normal with a remarkable improvement of headache. Epidermoid cysts involving the fourth ventricle have been known to be one of the most rare diseases in the central nervous system. This rare case is discussed and a review of the relevant literature is presented.