Even though pulmonary involvement is not widely appreciated by clinicians, an obstructive lung disease (specifically of the small airways) is another recognized component of Fabry disease. The original patient described by Fabry in 1898 had “asthma” and frequent respiratory tract infections [8] and died at age 43 of lung disease [9]. Several reports describe signs and symptoms of pulmonary involvement such as dyspnea, wheezing, and dry cough in Fabry disease [[10], [11], [12], [13]]. Many of these signs and symptoms were described without simultaneous cardiac compromise. Less frequent signs like haemoptysis, pneumothorax and pulmonary thromboembolism have been described.