This report described the clinical and pathological aspects of open spina bifida and diplomyelia along with multiple congenital malformations in a Texel lamb. Clinically, paresis of the thoracic limbs, paralysis of the pelvic limbs and a cutaneous opening in the lumbosacral region were observed. At necropsy, there was a focally extensive disruption of the skin associated with an absence of the dorsal portions of the lumbosacral vertebrae. Additionally, diplomyelia of the lumbar segment, mild hydromyelia of thoracic segment, and moderate communicating hydrocephalus of the lateral and third ventricles were noted. Possible viral etiologies (bovine viral diarrhea virus, bluetongue virus, and Schmallemberg virus) were not detected by RT-PCR, and toxic plants were not identified. Therefore, a possible genetic cause may not be discarded.