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Patients with arteriovenous malformations (AVMs) are at risk of significant hemorrhage and AVMs are especially difficult to manage in those desiring future pregnancy. Few patients with successful deliveries have been previously reported.The authors report an unusual case of AVM of the tongue in a pregnant woman who presented with massive pulsatile bleeding from a ruptured artery in the tongue in late gestation, this was thought to be caused by the changes in hormonal balance and the increase in cardiac output. The bleeding was controlled with transarterial embolization and transmucosal absolute ethanol sclerotherapy. Furthermore, her second and third deliveries were successfully managed.The authors managed symptomatic tongue AVM by combining transarterial embolization and transmucosal sclerotherapy, which was followed by successful deliveries. This patient supports the utility of transmucosal absolute ethanol sclerotherapy for tongue AVM and multidisciplinary medical care for a successful delivery.