A nine-year-old, neutered female beagle was presented with a history of progressive myoclonic jerks. Clinical signs included mental depression and paroxysmal jerks of the head and forelimbs, apparently elicited by changes in light, noise or movements. Electroencephalographic findings were in accordance with myoclonus epilepsy. Postmortem histopathological findings included multiple periodic acid-Schiff-positive inclusion bodies throughout the central nervous system, but especially in the cerebellum, confirming the suspected diagnosis of Lafora's disease.