A case of congenital segmental spinal hypoplasia was described in a 40-day-old Holstein calf. The calf was submitted to our clinic (Istanbul University, Faculty of Veterinary Medicine, Education and Research Hospital, Surgery Clinic) with the complaint of not being able to rise to feet after birth. After clinical and radiologic inspection an anomaly was detected in spinal cord at the level of 3rd and 4th lumbar vertebrae. As the prognosis of the animal deteriorates, the calf was euthanized and systemic necropsy of the animal was performed. There was no malformation at the vertebrae around lumbar region, but a constriction was found at the third and fourth lumbar region of the spinal cord in macroscopy. It was detected that the sections prepared from the narrowest part of the medulla spinalis revealed the entire disappearance of the canalis centralis, microscopically. Furthermore syringomyelic cavities with different dimensions were observed in dorsal and ventral funiculi.