Objective: To improve the understanding of clinical manifestations and imaging features of Sjögren’s syndrome myelopathy (SSM).

Methods: A case of SSM was reported. The clinical manifestations, etiology, imaging features and treatment principles of SSM were discussed with literature review.

Results: One elderly male patient complained mainly of limb weakness and diuresis difficulty; magnetic resonance imaging showed myelitis, and serum anti-nuclear antibody screening, ophthalmic dry keratoconjunctivitis and lip biopsy pathology confirmed SSM. The patient was transferred to department of rheumatology for treatment with glucocorticoid and rituximab, and the symptoms were relieved. 

Conclusion: Instead of the typical symptoms of dry mouth and dry eyes, the neuropathy included peripheral and central nervous system is often observed as the early manifestation in many Sjögren’s syndrome patients. Therefore, for the clinical diagnosis of Sjögren’s syndrome, attention should also be paid to the possible concomitant neuropathy. The incidence of SSM is not high, and it is easy to be misdiagnosed or missed. Therefore, differential diagnosis should be made between SSM and myelopathy, optic neuromyelitis spectrum disease and multiple sclerosis, so as to make a correct diagnosis as soon as possible, and give appropriate individual treatment.