Fabry disease is a lysosomal disorder caused by a deficiency or lack of α-galactosidase A (GLA). This leads to the histopathological accumulation of globotriaosylceramide (Gb3) and related glycoshingolipids within lysosomes in multiple cell types throughout the body, particularly cardiomyocytes, endothelial cells and vascular smooth-muscle cells in the heart [1]. Within the coronary vasculature, accumulation of Gb3 and related glycoshingolipids within endothelial and vascular smooth muscle cells causes structural abnormalities and progressive dysfunction. The resultant ischemia with non-obstructive coronary arteries (INOCA) is an increasingly recognized clinical entity with significant prognostic implications [2]. Vasospastic angina and coronary microvascular dysfunction (CMD) are important features of Fabry disease [[3], [4], [5], [6], [7]]. Although we previously reported a case of Fabry disease accompanied by Gb3 accumulation in capillary pericytes in the heart [8], at that time the functional impact remained unclear. The patient subsequently developed left ventricular hypertrophy and angina, despite receiving enzyme replacement therapy (ERT). Here, we report the findings of the same patient’s catheter examination, including a second endomyocardial biopsy (EMB), performed to assess the progression of the cardiac involvement in his Fabry disease, and propose a possible correlation between INOCA and pericyte impairment.