A 14-wk-old female llama (Lama glama) developed progressive neurologic disease characterized by stiff gait, circling, decreased mentation, and seizures. At necropsy, lesions were limited to the brain and consisted of bilateral necrosis of the cortical gray matter of the occipital lobes of the cerebral cortex. The primary microscopic alteration was bilateral laminar cerebrocortical necrosis, affecting mainly the deep laminae. Clinical disease, and gross and microscopic lesions were consistent with those of polioencephalomalacia.