Triglyceride deposit cardiomyovasculopathy (TGCV) is a novel clinical entity we found in patients withThese results suggest that TGCV might be difficult to diagnose in daily clinics, however be more prevalent than expected, and a possible important cause of cardiac death. This review article focuses on imaging modalities to evaluate cardiomyovascular TG accumulation of this novel disease. Data and images are shown from pathological analyses, imaging mass spectrometry, myocardial scintigraphy with a radioactive analogue for LCFA, iodine-123-β-methyl iodophenyl-pentadecanoic acid, MR spectroscopy, and CT-based TG imagings in primary TGCV patients with genetic ATGL deficiency, which is ultra-rare, but the monogenic model.