Children with Hereditary Hemorrhagic Telangiectasia (HHT) may have pulmonary arteriovenous malformations (AVMs), which can lead to symptoms of shortness of breath, exercise intolerance, clubbing, cyanosis and hemoptysis. However, some patients with pulmonary AVMs may be asymptomatic, placing them at risk for complications such as stroke or brain abscess if they are not identified and treated. This study examines the incidence of signs and symptoms associated with pulmonary AVMs in children with HHT known to have pulmonary AVMs. Sixty-one children with HHT and documented pulmonary AVMs were questioned for any symptoms possibly associated with their pulmonary AVMs, prior to embolization. The results show that the majority of these children were asymptomatic (56%), and their AVMs were discovered by routine screening. Those who did experience symptoms most often complained of shortness of breath and exercise intolerance, although there was a tendency to blame other conditions, such as asthma or deconditioning (being out of shape), for these symptoms. Migraine headaches were common in those with pulmonary AVMs, regardless of whether they were symptomatic or not (17 of 61 children, 28%), although they were more common in those who were symptomatic (10 of 27, 37%) versus those who were asymptomatic (7 of 34, 21%). Children with HHT and pulmonary AVMs are often asymptomatic. Routine screening for the presence of AVMs should be performed regardless of symptomatology.