Abstract Periventricular nodular heterotopia is a common neuronal malformation in humans, often leading to epilepsy and other neurologic diseases. A 2-month-old female Chihuahua weighing 750 g was examined because of a history of epileptic seizures and abnormalities in gait and behavior. Results of the clinical examination were consistent with a multifocal neurologic disease with localization in the forebrain and spinovestibular system. The magnetic resonance imaging showed multiple bilateral periventricular nodules isointense to gray matter and ventriculomegaly. Histopathological and immunohistological examination of the brain revealed that periventricular nodules consisted of neurons, fewer astrocytes, and some oligodendroglia consistent with periventricular nodular heterotopias.