The presented case is a 13-year-old boy with Rendu–Osler–Weber syndrome. He first presented with asymptomatic peripheral cyanosis at the age of 5 years 10 months, with echocardiography revealing no intracardiac anomaly. Screening computed tomography showed bilateral multiple arteriovenous malformations. The first coil embolisation of the largest malformation in the inferior portion of the left upper lung was performed at 7 years 2 months, with improvement in O₂ saturation from 81% to 93% achieved. The second coil embolisation for the residual shunts was performed 2 months later. After 4 years of uneventful life, he suffered four episodes of syncope and haemoptysis from 12 to 13 years of age. Further embolisation using more and more coils was performed during each episode until stable clinical condition was finally achieved. Rendu–Osler–Weber syndrome is an autosomal dominant hereditary disease. It is frequently associated with pulmonary arteriovenous malformation. Due to the high incidence of neurological complications in untreated patients, therapeutic embolisation is beneficial.

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Three-dimensional reconstruction of the pulmonary arteries showing multiple pulmonary arteriovenous malformations totally/partially/not occluded by coils (in green). Small diagrams (clockwise from upper left) represent: corresponding plain chest roentgenogram; left pulmonary artery angiography; and transverse computed tomography image at the level of the partially thrombosed largest arteriovenous malformation.