Sneddon’s syndrome (SS) is characterized by livedo racemosa (LR) or reticularis and recurrent ischemic strokes. At the skin and brain level a non-inflammatory thrombotic vasculopathy is observed. Almost 80% of cases are women around 40 years old. The most accepted etiological proposal is an autoimmune and inflammatory mechanism versus the presence of thrombophilia. Neurological manifestations occur in 3 phases: prodromal symptoms (headache, dizziness, and vertigo), recurrent strokes, and early-onset dementia. Livedo racemosa has been reported to precede strokes by more than 10 years. Treatment is mainly based on secondary prophylaxis preventing a stroke with antiplatelet and antithrombotic agents. The neuropsychiatric prognosis is relatively poor with deficits in concentration, attention, visual perception, and visuospatial skills.