Abstract: The authors reported a case of cardiac epithelioid angiosarcoma (EA) with extensive metastasis on ¹⁸F-fluorodeoxyglucose (FDG) PET/CT imaging. The characteristics of EA were analyzed from clinical symptoms, laboratory examinations, ultrasound, CT, MRI and ¹⁸F-FDG PET/CT whole-body imaging, and the imaging features of cardiac epithelioid angiosarcoma were summarized through literature review. Cardiac EA is rarely seen in clinic, and ¹⁸F-FDG PET/CT imaging has certain clinical significance in the diagnosis and clinical staging of EA.