There is limited evidence regarding temporary discontinuation of ERT in Fabry disease. Cabrera and Politei [26] reported one male who started agalsidase beta. After two years of favorable response to ERT, the patient decided to suspend treatment. Patient presented two years later with increasing pain, fatigue, progression of cardiac involvement and new white matter lesions in brain MRI. Treatment discontinuation resulted in a fast deterioration of impaired organs and the appearance of new Fabry manifestations.