The death of a 6-month-old, male, white lion with micrognathia and short, thick limbs in a zoo prompted postmortem whole-body computed tomography and head magnetic resonance imaging to be performed. Computed tomography revealed malformation of all bones. The metaphyses of the long bones were enlarged and showed fraying and cupping. Moreover, magnetic resonance imaging revealed abnormal bone tissue with reduced bone density, severe bilateral compression of the spinal cord from the stomach up to the level of the atlas and herniation of the cerebellar vermis. Histopathological examination revealed that the metaphyses of all long bones were enlarged and replaced with cartilage; however, the structure of the joints was maintained. This report presents the first case of congenital systemic chondrodysplasia identified by postmortem examination and imaging in a lion.