A newborn Thoroughbred foal was presented to the clinic with ambiguous neurological deficits, spinal anomalies and a soft tissue swelling dorsal to the lumbar vertebral column. The foal was alert but unable to rise and stand. With radiography, ultrasonography, computed tomography (CT) and magnetic resonance imaging (MRI) a lumbar dysraphic anomaly, cerebellar herniation and coincidental skeletal abnormalities were documented. Finally, a meningomyelocele was defined and, in context with the cerebellar herniation through the foramen magnum, the foal was diagnosed to have a Chiari malformation. The MRI examination corresponded best with the post mortem findings. Although 3-dimensional imaging methods have been considered superior regarding full and detailed assessment of the congenital malformation, radiography and ultrasonography also provide essential information to diagnose dysraphic lesions at reduced costs and efforts. A Chiari malformation should be considered as a differential diagnosis in foals with neurological deficits.