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Osler–Weber–Rendu syndrome is a disease of hereditary telangiectasia with involvement of multiple visceral organs. Pulmonary arteriovenous malformation (AVM) is a frequent clinical manifestation, that may mimic pulmonary mass. In this report, endobronchial ultrasound (EBUS) was used in a 64-year-old woman with Osler–Weber–Rendu syndrome who had a solitary pulmonary mass associated with clinical evidence of a right-to-left shunt and was able to detect a pulsating hypoechoic mass connected to the right pulmonary artery suggesting of pulmonary AVM. Following magnetic resonance angiogram and chest CT as well as noting multiple telangiectasis on her skin and considering a strong family history, Osler–Weber–Rendu syndrome was confirmed. Exercise intolerance and desaturation improved after surgical resection of the pulmonary AVM. The echoic findings were correlated to the pathological features. EBUS can be used as a good diagnostic tool for determining the nature of peripheral lung lesions such as in this case, and EBUS may be of practical tool to investigate a lung mass when a vascular component is suspected.