Six patients with a variant type of Jansky-Bielschowsky (JBVD) disease were examined using 3 different imaging methods. Five of the patients underwent computed tomography, 4 magnetic resonance imaging, and 5 single photon emission computed tomography. All patients had brain atrophy that was most severe in the cerebellum. Magnetic resonance imaging demonstrated the parenchymal abnormalities well; all patients had hyperintense periventricular white matter, especially around the bodies and atria of the lateral ventricles, and a significant decrease in signal intensity in the thalami and/or putamina. Single photon emission computed tomography disclosed hypoperfusion of the cerebellum in all patients. Neuroimaging examinations are valuable in the diagnosis of JBVD. It may be difficult to divide patients with neuronal ceroid-lipofuscinosis disorders into clinical subtypes in the early stage of the disease. Magnetic resonance imaging, especially when combined with a typical clinical pattern, makes the diagnosis of JBVD highly likely. Radiologic examinations of the brain may also prove important in following the progression, as well as in investigating the pathophysiology, of the disease.