Primary cardiac sarcomas are exceptionally rare tumours. A brain metastasis of a primary cardiac sarcoma has never been reported before. Although we know lots of primary malignomas spreading to the brain, we never observed cerebral metastases of an atrial spindle cell sarcoma. Cardiac sarcomas are more likely to haematogenously metastasize to the lung or the liver. Here, we describe the case of a young man, who suffered from a cerebral metastasis of a spindle cell sarcoma in the left heart atrium nine years ago. Postoperative whole brain irradiation with 30 Gy was performed. Later on, the patient experienced a local recurrence within the left atrium accompanied by cardiac arrhythmia and mitral valve insufficiency. This case is the very first description of a true cerebral metastasis from a primary heart sarcoma. Therefore, clear treatment paradigms are not established. Further case illustrations and the publication of larger patient series are mandatory, whenever possible.