Left atrial myxomas are a rare stroke etiology (0.4%). They frequently present general symptoms such as fever, weight loss and an inflammatory syndrome, but the pseudolupic syndrome with cutaneous features and vasomotor symptoms is rarely described. We report the case of a 38-year old man who presented in March 1995 a stroke caused by a left atrial myxoma. There was also pseudolupic clinical and biological manifestations which disappeared after ablation of the myxoma. Cerebral angiography showed a small carotid aspect which evoked carotid dissection or carotid siphon occlusion. The authors discuss neurologic and pseudolupic manifestations of left atrial myxomas, and their physiopathologic mechanism.