The authors report an unusual case of left atrial myxoma in a 30-year-old woman, discovered after a recurrent stroke. This tumor was misdiagnosed earlier because of an exclusive neurologic symptomatology, a normal cardiac exam without any sign of mitral obstruction (unusual high implantation of the myxoma within the roof of the left atrium), and the lack of doing an echocardiography which should be systematically done after an ischemic stroke, even if its etiology seems to be evident. Surgical resection of the tumor led to prevent further myxomatous emboli, but unfortunately, the patient keeps severe neurological sequelae.