Benign lesions that present as neck masses are relatively common findings i
n the pediatric otolaryngology practice. The differential diagnosis include
s persistent fetal structures, such as thyroglossal duct and branchial clef
t remnants, as well as vascular malformations, such as hemangiomas and arte
riovenous malformations. Persistent fetal vascular structures, however, are
less common. We describe a 2-year-old boy who presented with a mass in the
anterior aspect of his neck that was determined to be a persistent anomalo
us fetal vein. We also discuss the embryology and development of the venous
system in the neck, as well as the options for the diagnosis and managemen
t of benign vascular lesions that present as neck masses in the pediatric p
atient.