Abstract
Congenital hepatic arteriovenous malformations (HAVMs), though rare, carry high morbidity and mortality rates if left undiagnosed. The usual clinical presentation is in infancy with congestive heart failure, anaemia and hepatomegaly. There are reports of presentation as persistent pulmonary hypertension in newborns and reports of their spontaneous regression as well. We describe a healthy full-term neonate with HAVM who was presented with isolated massive hepatomegaly and underwent surgical ligation.