BackgroundCardiac inflammatory myofibroblastic tumor (IMT) is an extremely rare benign entity which constitutes a small proportion of primary cardiac tumor.Case presentationWe present a rare case of a 55-year-old symptomatic male patient with a rare cardiac IMT causing left ventricular outflow tract (LVOT) obstruction. The patient received complete tumor resection immediately with uneventful postoperative hospital course.ConclusionTo our best knowledge, cardiac IMT involving aortic valve in older adult causing LVOT obstruction has never been reported before. Additionally, we make a literature review on IMT, focusing on the various clinical presentation of this unpredictable tumor.