A 61-year-old male presented with a hypervascular variant of dysplastic gangliocytoma (Lhermitte-Duclos disease) manifesting as gait disorder. Computed tomography and magnetic resonance imaging both showed enhancement of the tumor after injection of contrast medium. Angiography demonstrated a tumor stain. Histological examination showed a double-layered structure comprising an outer layer of myelinated axons and an inner layer of dysplastic granular cells, and numerous dilated thin-walled blood vessels. Partial resection of the tumor resulted in resolution of the neurological deficit.