Primitive Gougerot-Sjögren syndrome (PGSS) is a systemic autoimmune exocrinopathy in which twenty percent of affected patients develop neurological manifestations. It has been little described in the Black African literature. We report a case of PGSS in a 64-year-old Black woman as revealed by neurological manifestations in an inflammatory context. Central neuropathy was present and the patient fulfilled at least four of the six Vitali American-European diagnostic criteria. There were abnormal aspects on the sylvian artery and hyperintense lesions of periventricular predominance. PGSS is a serious disease which we must consider when we encounter any unexplained neurological manifestations especially those associated with vasculitic lesions.