Autoimmunity can manifest clinically in many ways; however, despite the various efforts to classify autoimmune disorders into specific disease entities, the borders between these disorders remain, in many cases, unclear. In this report we describe a young woman with subclinical Sjögren's syndrome and biliary cirrhosis, who presents clinically with symptoms exclusively from the central nervous system. This neurological syndrome is consistent with a progressive myelopathy. Although the patient has a serologically and histologically confirmed multisystemic autoimmune disorder, she fulfills none of the classification criteria for the diagnosis of a specific connective tissue disease.