Abnormalities of the nervous system are common occurrences among congenital defects and have been reported in most ruminant species. From a clinical standpoint, the signs of such defects create difficulty in arriving at an antemortem etiology through historical and physical examination alone. By first localizing clinical signs to their point of origin in the nervous system, however, a narrower differential list can be generated so that the clinician can pursue a definitive diagnosis. This article categorizes defects of the ruminant nervous system by location of salient clinical signs into dysfunction of one of more of the following regions: cerebrum, cerebellum,and spinal cord. A brief review of some of the more recognized etiologies of these defects is also provided. It is important to make every attempt to determine the cause of nervous system defects because of the impact that an inherited condition would have on a breeding program and for prevention of defects caused by infectious or toxic teratogen exposure.