Treatment of Fabry disease has improved since the introduction of enzyme replacement therapy (ERT). Two preparations of the recom binant enzyme α‑galactosidase A are available: agalsidase alfa and agalsidase beta. We aim to report on a disease improvement after switching therapy from agalsidase alfa to agalsidase beta in a patient with Fabry cardiomyopathy. We present a case of a 60‑year‑old male with chronic renal failure and hypothyroidism, diagnosed with Fabry disease in 2010. We investigated clinical changes in this pa tient during the 12‑months follow‑up. At the time of diagnosis, transthoracic echocardiogram (TTE) and cardiac magnetic resonance (CMR) showed widespread transmural inferolateral late enhancement (LE), poor ejection fraction and severe left ventricular hypertrophy. Despite of initiation of ERT with agalsidase alfa, clinical status of the patient did not improve. A shift to agalsidase beta was made, what resulted in marked betterment. Effectiveness of ERT on Fabry cardiomyopathy primarily depends on the stage of disease at baseline, since ERT may provide limited benefits in patients with evidence of fibrosis in CMR. Switch from agalsidase alfa to agalsidase beta may be needed in some patients, who do not improve on the first‑line therapy.