Arteriovenous malformation in a patient with Bannayan--Zonana syndrome

Bannayan-Zonana syndrome (BZS) is a genetic disorder with autosomal dominant inheritance characterized by macrocephaly and multiple hamartomas of mesodermal origin. Here we present a patient with BZS manifested by many of the classic features, as well as a high-flow upper extremity arteriovenous malformation (AVM). Although this rare syndrome was initially described in 1971, to our knowledge, this is the first report showing an association of AVM with BZS and the first report of this syndrome in the radiologic literature.