ABSTRACT: A case of lissencephaly-pachygyria and cerebellar hypoplasia diagnosed in a Charolais x Tabapuã calf is described. The calf presented since birth, clinical signs characterized by apathy, prolonged recumbency, tremors of the head and neck, ataxia, hypermetria, difficulty walking, blindness and swelling of the joints of the four limbs. Due to the unfavorable prognosis, the animal was euthanized and necropsied at 34 days of age. At necropsy, a rudimentary development of the brain folds (gyri) and grooves (sulci) was observed, and the cerebellum was hypoplastic. The cut surface of the brain exhibited thickening of the gray matter (pachygyria) in the frontal, parietal, temporal and occipital cortices and narrowing of the white matter. In the organs of the thoracic and abdominal cavities, no significant lesions were observed. Histologically, cerebral cortex was thick and exhibited neuronal disorganization of the gray matter. The cerebellum had a thin molecular layer, and neuronal disorganization with ectopia of the Purkinje neurons in the region of the granular and molecular layers. There were no bacterial growths in cultures of joint swabs. This was the only case on the property, which suggests that this malformation, which has not previously been described in cattle, was a sporadic case, and it was not possible to determine its cause. Neurological lesions and clinical sings presented here should be considered in the differential diagnosis of congenital diseases of the central nervous systems of cattle.