Conditions of the equine caudal neck (C5-C7) are thought to be associated with various clinical signs. However, little investigation has been undertaken to isolate clinical indicators associated with specific conditions of the equine caudal cervical spine. This systematic review aimed to evaluate associations of clinical signs with anatomical malformation of caudal cervical vertebrae, spinal cord compression of the caudal cervical spine, and arthropathy of the caudal cervical articular process joints (APJs). A literature search was carried out using Google Scholar in accordance with PRISMA (Preferred Reporting Items for Systematic reviews and Meta-Analyses) guidelines. Studies were selected for inclusion in this systematic review according to a set of inclusion criteria, resulting in a small group of eligible studies (n = 12) that addressed clinical signs associated with caudal cervical spine conditions in horses. The results of the included studies indicate that there are grounds for further investigation of clinical presentations of specific conditions of the equine caudal neck such as anatomical variations, myelopathy, and alteration to the APJs.