Fabry disease (FD) is a rare, multisystemic X-linked inherited lysosomal storage disorder, in which the deficient activity of the enzyme α-galactosidase A leads to the accumulation of globotriaosylceramide in affected tissues in the body. Cardiac involvement in Fabry disease occurs as a part of the classic, early-onset variant of the disease, more commonly seen in men, as well as in the cardiac or atypical, late-onset variant of the disease. Cardiovascular involvement can manifest as a left ventricular hypertrophy, myocardial fibrosis, valve disease, heart failure, arrhythmias, coronary complications and sudden cardiac death. These complications carry a major prognostic impact on Fabry disease, representing the main cause of death in patients. Most commonly used diagnostic methods in evaluation of cardiac involvement include electrocardiography, echocardiography, cardiac magnetic resonance imaging and laboratory biomarkers. Advances in cardiac imaging techniques have improved the diagnosis and staging of Fabry cardiomyopathy, while with the better understanding of pathophysiology of the disease, the FD-specific treatment landscape is evolving rapidly. The main goal of the treatment is to prevent disease progression and irreversible organ damage. Currently, the main approved FD-specific treatments include enzyme replacement therapy (ERT) and the pharmacological chaperone migalastat. If instituted early in the disease course, the treatment has shown to achieve a better outcome and might slow the disease progression,while also having a positive effect on cardiac complications. Therefore, early recognition and treatment are crucial. Cardiac complications are treated with conventional treatment by the current guidelines, incorporating a few caveats considering the very nature of Fabry's disease. The cooperation of various specialists and experts as well as a holistic approach to treatment are essential for optimal care of these patients who require lifelong monitoring. The goal of this thesis is to provide a comprehensive overview of current knowledge on the pathophysiology, diagnosis, approach and the treatment of cardiac complications of Fabry disease.