Livedo racemosa with cerebrovascular lesions has been described as Ehrmann-Sneddon syndrome. The etiopathogenetic factors provoking the vascular lesions, however, are of high diversity reaching from mechanical to autoimmune causes. We present a male patient with typical livedo racemosa, muscle pain and feeling of coldness of the forearms. By dermatohistopathology and magnetic resonance tomography of the brain, Ehrmann-Sneddon syndrome could be confirmed. At this time a chronic streptococcal infection could be diagnosed. Antibiotics, anticoagulants and vascularity-supplying therapy improved the clinical and subjective symptoms. Six months later, the patient developed dizziness, vision disorder, hypesthesia of the right forehead, malaise and weight loss. A further diagnostic workup including echocardiography revealed a myxoma of the left atrium. This report illustrates the association of Ehrmann-Sneddon syndrome with cardiac myxoma and points out that cardiac diagnostic examination should be included when dealing with small-vessel involvement of the brain.