In this report a case of prosencephalic hypoplasia in a stillborn calf is described. It is characterized by the absence of the cerebral hemispheres, malformation of the diencephalon and cerebellum, and an abnormally flattened calvaria with cranioschisis and encephalocele. The absence of the telencephalon is also typical of anencephaly and hydranencephaly, from which prosencephalic hypoplasia has to be differentiated. These three types of congenital malformations of the bovine brain are briefly discussed with special focus on their morphologic characteristics in order to allow a specific diagnosis.