Objective To investigate the clinical features, therapeutic strategies, and prognostic outcomes of hepatic hemangioma with arteriovenous fistula (HH-AVF) in neonates. Methods Clinical data of 8 neonates diagnosed with HH-AVF and admitted to our institution between January 2018 and December 2024 were retrospectively collected. Their clinical manifestations were analyzed, with the efficacy of interventional therapy evaluated. Results Among the 8 neonates with HH-AVF (5 males and 3 females), all presented with hepatic space-occupying lesions. Six cases developed respiratory distress requiring mechanical ventilation, with chest radiography revealing cardiomegaly and complications including heart failure and pulmonary hypertension. Two cases had multiple cutaneous hemangiomas, and one case had a cervical hemangioma with bleeding. All 8 neonates underwent interventional therapy at a median age of (29.5±9.5) days, combined with oral propranolol. One neonate showed an increase in hepatic mass on follow-up ultrasound at 4 months post-operation, prompting a second interventional embolization due to suspected tumor progression; the remaining 7 cases improved and were discharged. During a follow-up period of 12-24 months, none of the patients experienced recurrence of respiratory distress, pulmonary hypertension, or heart failure, and all lesions were significantly reduced or resolved compared to the time of diagnosis. Conclusions Neonatal-onset HH-AVF exhibits diverse clinical manifestations, with higher mortality rates in cases complicated by heart failure and pulmonary hypertension. Interventional therapy may be an effective approach to improve the prognosis of HH-AVF.