Congenital brain defects in cattle are usually obvious at birth, but detection may depend on the nature and extent of the defect. The cause may be genetic or environmental, or the cause may be unknown. Many central nervous system defects are inherited as a simple autosomal recessive trait and may have a significant economic impact on purebred and commercial cattle operations. A common structural defect of genetic nature is internal hydrocephalus. A functional defect of simple autosomal recessive inheritance is bovine progressive degenerative myeloencephalopathy. The most significant teratogenic agents causing central nervous system defects are prenatal viral infections such as bovine virus diarrhea and bluetongue viruses. Many other defects of the central nervous system are reviewed.