Cardiac manifestations of Anderson-Fabry disease are usually part of a multiorgan involvement; they are frequently recognized in the young adult and increase morbidity and mortality. Cardiac complications, first hypertension and diastolic dysfunction, are observed in about half of patients and are accessible to the usual management of hypertension, heart failure, coronary artery disease, rhythm or conduction disturbances. However, the cardiac variant may present as an isolated or predominant cardiac involvement, with left ventricular hypertrophy being the most apparent sign, that could lead to the wrong diagnosis of « idiopathic » hypertrophic cardiomyopathy of sarcomeric origin (in 1-4% of cases, up to 6% in males before 40 years). However, in Fabry disease, hypertrophy is more often concentric without subaortic obstruction, while search for signs of Fabry disease (history of acroparesthesia or anhidrosis, renal dysfunction or stroke) should be systematic. Early identification of subjects with Fabry disease allows to check for target organ damage, family screening, genetic counseling and specific enzyme replacement therapy. The latter, in the absence of irreversible and extended myocardial fibrosis and/or severe renal dysfunction, is efficient on the progression of renal disease and cardiac hypertrophy and delayed in parallel the occurrence of a first renal, cardiac or neurologic event.