Intradiploic epidermoid cysts of the cranium are rare, benign and slow growing lesion. However, these cysts may cause neurologic deficits due to mass effect. Intradiploic epidermoid cysts are thought to be derived from persistent ectodermal remnants present in the cranial bones during embryogenic development. Here, we report a case of an occipital intradiploic epidermoid cyst with posterior fossa extension. The patient developed intracranial hypertension associated with hydrocephalus due to aqueductal stenosis, and venous congestion secondary to compression of the torcular and the transverse sinus. The imaging studies included a computerized tomography[CT] venogram, which is essential for determining the pathophysiological mechanism of the clinical spectrum and for surgical planning. Near total removal was accomplished and the postoperative course was uneventful. Postoperative imaging studies demonstrated a reversal of the neural structures, but no patency of the sinus, which was presumably indicative of prolonged sinus stenosis. The patient gradually improved and was discharged free of symptoms.