Two cases of Lhermitte-Duclos disease confirmed by biopsy are reported. Review of the 58 published cases shows that the disease can manifest itself only by signs of increased intracranial pressure. Cerebellar symptoms are not constant. Computed tomographic (CT) scans suggest the diagnosis by showing a posterior fossa lesion, iso- and hypodense, partially calcified, and not enhanced by contrast medium. Magnetic resonance imaging (MRI) seems to define limits of the lesion better than CT scanning and could improve the surgical approach. Surgical excision of the lesion is the only satisfactory treatment. The postoperative prognosis is usually favorable.