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Syringomyelia is associated with more than two-thirds of Chiari malformation type I cases, and rarely with intramedullary neoplasm or post-traumatic cavitations. Some authors assume that syringomyelia, sometimes observed in patients with multiple sclerosis, is more likely to be a consequence of inflammatory-demyelinating pathology of the spinal cord. We describe a case of a 23-year-old patient who was diagnosed with multiple sclerosis according to the McDonald criteria with syringomyelia and Chiari malformation type I. It to be a very rare case of co-occurrence of syringomyelia and multiple sclerosis as two separate unrelated independent diseases.