One case of an unusually giant epidermoid cyst of the skull is described. The neoformation had been present for 40 years, becoming extremely large in the last 20 years, in a 65-year-old woman. Difficulty in wearing glasses was the sole complaint of the patient, who otherwise concealed the mass by wearing a wig. Preoperative examinations (x-rays, CT scan, MRI) were important to better evaluate the nature of the cyst and to make a differential diagnosis. Dissection of the mass from the surrounding tissues was extremely easy because of its capsule. Sixteen months of follow-up have been pleasant, with no evidence of recurrence.