Epidermoid cysts are uncommon accounting for less than 1% of all intracranial tumors. Among them, giant intradiploic epidermoid cysts of the posterior fossa i.e. those exceeding 5 cm are an even rarer entity. Here we report on the complete resection of a 7 cm posterior fossa epidermoid cyst involving the occipital bone in a 32-year-old female presenting with an acute on chronic exacerbation of headaches and vertigo. Complete resection including the cyst wall is crucial in preventing recurrence and malignant transformation. We add to limited existing data by reporting on the first systematic review to date assessing 25 cases of giant epidermoid cysts of the posterior fossa to discuss presentation, clinical and radiographic features and definitive management strategies.
