Abstract
BACKGROUND: Malignant tumours of the aortic valve apparatus are extremely rare and difficult to diagnose. Their proximity to the coronary ostium may cause an acute coronary syndrome (ACS) either by infiltration or by embolization. CASE SUMMARY: We report a case of primary aortic valve undifferentiated sarcoma causing recurrent episodes of ACS, and we provide a literature review for primary cardiac valve tumours. This case also highlights the need for further evaluation of other causes of ACS in patients with minimal coronary artery disease risk factors and recurrent ACS. CONCLUSIONS: The majority of valve tumours are fibroelastomas. Sarcomas are rare and lead to poor outcomes.