Pulmonary arteriovenous malformations (PAVMs) are a rare clinical entity. Most of them are associated with hereditary haemorrhagic telangiectasia. The usual clinical presentation is exertional dyspnoea and hypoxaemia. The initial test of choice for screening is the 100% oxygen method. A pulmonary angiogram is needed to define the anatomy and guide transcatheter embolisation (TCE). TCE has been shown to be effective and safe with a very low recanalisation rate and has largely replaced surgery for PAVMs. Computed tomography of the chest can be used for the follow up of asymptomatic PAVMs and TCE.