Chiari-like malformation represents a congenital anomaly that affects the bony cranial base and the hindbrain, leading to fluid filled cavities formation in the spinal cord, condition named Syringomyelia. This paper aims to assess the variety of the clinical signs and to evaluate the magnetic resonance imaging findings in thirty- seven Cavalier King Charles Spaniel dogs. The study was performed over a four-year period, from 2013 to 2017, all cases underwent neurological examination, full diagnostic work-up, including magnetic resonance imaging scans of the brain, cervical and upper thoracic spinal cord. Thirty-seven dogs were included in this study, 23 females and 14 males, with a mean age of 3.6±2.1years. The commonest clinical findings encountered were neuropathic pain and vocalization, seen in all 37 cases, followed by scratching, facial rubbing, paw licking, air licking, tail chasing, seizures and unilateral facial paralysis. Different grades of cerebellar herniation and cervical syrinxes were noted in all cases, other magnetic resonance imaging findings encountered were medullary kinking, presyrinx and ventriculomegaly. In establishing the diagnosis of the Chiari-like malformation and Syringomyelia, the breed, clinical history and the symptomatology are very important, but only magnetic resonance imaging technique can provide quantitative assessment of the nervous system lesions.