Concurrent occurrence of occipital dysplasia and occipital hypoplasia in two dogs is described in this report. Occipital hypoplasia results in reduced volume of the caudal fossa, leading to overcrowding of the neural structures and, in severe cases, development of syringomyelia. In occipital dysplasia, there is a failure of complete ossification of the supraoccipital bone. When the two conditions occur concurrently, it is possible that syringomyelia may develop more slowly, resulting in presentation with clinical signs in middle to old age. This has implications for screening tests for early detection of syringomyelia, with a view to using the dog for breeding purposes, as dogs with an apparently mild phenotype for occipital hypoplasia/syringomyelia may actually have a more severe genotype.