Objective: To improve the understanding of Sneddon’s syndrome (SS) in clinical practice. 

Methods: The clinical diagnosis and treatment of a patient with SS complicated with splenomegaly and lymphadenectasis were reported. The clinical manifestations, diagnosis and possible mechanism of SS were discussed based on literature review.

Results: A SS patient firstly manifested recurrent ischemic cerebrovascular accidents followed by livedo racemosa of the skin, accompanying hearing impairment, mild cognition decline, headache and other symptoms. Anti-phospholipid antibody (APA) was negative. Imaging examination showed splenomegaly and lymphadenectasis. Skin biopsy found atypical pathological changes. After treatment with antiplete and circulation-improving drugs, symptoms of the patient were alleviated a lot. 

Conclusion: SS complicated with splenomegaly and lymphadenectasis is rare. The precise mechanism is unknown, may be related to some antibodies targeting endothelial system, which are different from anti-phospholipid antibodies (APAs) and could not be detected currently. When the symptoms of SS are atypical or unspecific, misdiagnosis or missed diagnosis is likely to happen. With recurrent attacks of stroke, the possibility of severe damage to neurologic
function and vascular dementia is increased. Therefore, early recognition of SS to make correct diagnosis and treatment as soon as possible is very important.