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Congenital neonatal scalp arteriovenous malformation: a very rare entity. Congenital arteriovenous malformations (AVMs) of scalp are rare congenital vascular malformations. They are usually not symptomatic at birth and are often misdiagnosed as haemangiomas. To date, only two cases of symptomatic neonatal scalp AVM have been reported in literature. Pathophysiology of congenital AVM is not completely understood but genetic and acquired causes are implicated. Diagnosis and management are often difficult and require multidisciplinary approach. We report a rare case of symptomatic congenital scalp AVM in a 10-day-old neonate who was successfully managed at our unit.