Neurologic dysfunction accompanied by malformation of both the skull and the cervical vertebrae has been previously described in lions kept in captivity worldwide, and this dysfunction and malformation were most often related to vitamin A deficiency. Diagnosis of the bone malformation and its effects on the neural tissue was until recently limited to postmortem examination, with characteristic thickening of the bones of the cranial vault, cerebellar herniation, compression of the foramen magnum, and enlargement of the lateral ventricles. For some mildly affected lion cubs with neurologic signs, improvement was reported with excessive vitamin A supplementation. However, definitive diagnosis was only available for those that eventually died or were euthanized. This case documents the antemortem diagnosis of the disease using computed tomographic imaging and liver biopsy. While conservative treatment failed, suboccipital craniectomy removed the thickened occipital bone and was demonstrated to be a successful surgical intervention that can be used to treat more severely affected lions.