We describe a patient with livedo racemosa generalisata (LRG), Raynaud's phenomenon, multifocal recurring transitory and complete ischemic attacks and laboratory findings of systemic inflammation. Working diagnoses were vasculitis, mixed connective tissue disease, or Sneddon's syndrome, but echocardiography revealed a left atrial myxoma. Special staining of skin biopsies with alcian-blue showed arterial occlusion caused by myxomatous material. Arterial embolism by parts of the myxoma is suggested to be the cause of the LRG and probably the neurological findings.