Abstract: OBJECTIVE To analyze a case of ventricular electrical storm following agalsidase alfa administration in a patient with Fabry disease, explore the causality assessment of the adverse reaction and clinical management strategies, and provide reference for ensuring medication safety. METHODS Clinical pharmacists, based on the temporal relationship of drug administration and exclusion of other triggers, assessed the causality between the adverse reaction and agalsidase alfa as "possible," and assisted in formulating a comprehensive treatment plan including discontinuation of agalsidase alfa, administration of amiodarone, and cardioversion. RESULTS The patient's arrhythmia was gradually controlled and the condition remained stable. Analysis suggested that agalsidase alfa may induce electrical storm through disturbance of myocardial enzyme metabolism or direct myocardial stimulation. CONCLUSION Although cardiotoxicity induced by agalsidase alfa is rare, it may be life-threatening. Clinicians should strictly evaluate the baseline cardiac status of Fabry disease patients before initiating enzyme replacement therapy, formulate individualized dosing regimens, strengthen electrocardiographic monitoring, and enhance awareness of adverse reaction recognition and prevention to ensure medication safety.