IntroductionThere are few reports on arrhythmia treatment in patients with the later-onset phenotype of Fabry disease (FD). Case reportAblation was performed for recurrent atrial fibrillation (AF) in a patient with FD. Even after the re-isolation of the left inferior pulmonary vein (LIPV), AF was easily induced. After fractionated signal area ablation, AF could not be sustained even with ganglionated plexi stimulation. Discussion FD is a rare disease that progressively causes myocardial degeneration, and PVI alone may be insufficient as an AF ablation strategy. Attempting non-PV substrate ablation in AF patients with FD may be worthwhile.