Myxoma-related intracranial diseases were rarely documented in history. The main purpose of our study is to provide a more comprehensive and detailed understanding of the pathogenesis, imaging features, surgical procedures and pathology of such patients through long-term follow-up. From March 2012 to July 2018, baseline information that included neuroimaging and neuropathology data from 12 cardiac myxoma patients with neurological symptoms were retrospectively analysed, and the treatment options were discussed. Nine patients underwent long-term postoperative follow-up. Twelve cardiac myxoma patients with neurological symptoms were identified, and among them, 10 patients were postoperative patients who had undergone excision of cardiac myxoma, 5 patients had received craniotomy, and the others had received conservative treatment. Positive neuroimaging findings were found in all patients, including cerebral infarction (12/12, 100%), multiple intracranial aneurysms (8/12, 67%), and extravascular metastasis (6/12, 50%). After a long-term average follow-up of 27 months, an increased number of metastatic lesions or an enlargement of the intracranial aneurysms was found in 4 patients. Neuroimaging findings of myxoma-related intracranial lesions were diversed and usually presented as multiple cerebral infarction, aneurysm formation, focal intracranial haemorrhage and space-occupying lesions. Progress is over a long period of time after primary tumour resection. It is necessary for patients to be regularly examined within 2 years after cardiac myxoma resection using MRI+CTA/MRA/DSA in order to be ruled out. Stable and effective chemotherapy drugs are urgently needed.