Two young (14-mo-old and 6-mo-old), unrelated, male African lions (Panthera leo) were presented to the Veterinary Teaching Hospitals of Oklahoma State University and Kansas State University with progressive ambulatory difficulty. In both cases, limited neurologic evaluation demonstrated pelvic limb paresis and ataxia with conscious proprioceptive deficits. Spinal imaging showed nearly identical lesions in both cases. Radiography and myelography demonstrated cervical stenosis secondary to atlantal (C₁) malformation producing a dorsoventral deformity of the laminar arch with atlantoaxial spinal cord compression between the ventrally displaced laminar arch of the atlas and the underlying odontoid process of C₂. Computed tomography of the atlanto-axial junction confirmed cervical stenosis and cord compression, showing flattening of the spinal cord between the laminar arch of C₁ and the dens of C₂. Decompressive surgery consisting of dorsal laminectomy of C₁ was performed. Each lion demonstrated progressive improvement of neurologic status to recovery of normal ambulation after surgical intervention. Neurologic disease in large captive felids is rare; atlanto-axial spondylomyelopathy has not been reported previously.