Cardiac myxoma is a rare but important cause of stroke, which affects young people. More recently the diagnosis has been enhanced by the use of echocardiograms. We aimed to review the neurological presentations, including stroke, of cardiac myxoma in this modern era of diagnosis and management. Records of patients with neurological presentations at the Austin and Repatriation Medical Centre and The Northern Hospital were retrieved from 1985 to late 2001, using International Classification of Diseases codes for atrial myxoma. Published literature reports were obtained by using Medline search database. An iterative process of bibliography review was utilised to identify reports not found by primary search. Case demographics, neurological presentations, investigations, treatment and outcome were recorded. From the Austin and Repatriation Medical Centre and The Northern Hospital, 6 cases were reported in detail and 107 cases from the published literature were analysed. The mean age of all cases was 43 (range 6-82). There was a female to male predominance (3:2). While there were overlapping neurological presentations, the most common presentation was ischaemic stroke (83% of all patients) most often in multiple sites (41%). The other presentations included syncope (28%), psychiatric presentations (23%), headache (15%) and seizures (12%). Commonest means of reaching the diagnosis was by echocardiography. The myxoma was surgically resected in 69% of cases. Of all cases, 24% were autopsy reports, almost all prior to availability of echocardiograms (in mid-1970s). Patients who presented with neurological complications of cardiac myxoma were young and stroke was by far the most common single presentation. Importantly, when all clinical manifestations were considered, almost half were potentially reversible. In recent years, echocardiography has made significant contribution to establishing the diagnosis less invasively. There is uncertainty about the role of anticoagulants. The treatment of choice remains surgical excision, although the timing post stroke is debatable. There is a need for large scale collaborative studies to help refine management strategies.