A man in his mid-40s, diagnosed with motor neuron disease (MND) and Fabry disease (FD), was sent to cardiology after experiencing a cardiac arrest at home secondary to ventricular fibrillation (VF). Fabry disease is a rare X-linked inherited lysosomal storage disorder caused by deficient alpha-galactosidase A (AGAL-A) activity that leads to an accumulation of globotriaosylceramide (Gb3) in affected tissues, including the heart. This evidence concerns the gene GLA and motor neuron disorder.