FD is a rare X-linked genetic disorder resulting from the deficiency of the enzyme α-galactosidase A. This enzyme deficiency leads to the accumulation of glycosphingolipids, particularly in vascular endothelial cells and smooth muscle cells. Cardiac involvement is a significant complication, often manifesting as cardiomyopathy, which can lead to serious morbidity and mortality. Patients often experience left ventricular hypertrophy (LVH), arrhythmias and heart failure. Cardiac MRI is a crucial imaging modality for the quantitative and qualitative assessment of FD cardiomyopathy.  Identification of characteristic CMR findings in patients with suspected or confirmed Fabry disease is vital to allow early diagnosis of disease, risk stratification to guide clinical management and monitor disease progression.