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Venous malformations (VM) of vulva, perineum and pelvis are uncommon condition which may present with cutaneous varices or aesthetically disabling swelling of external genitalia. Herein, we report a young woman who presented with a large left vulva bluish tinged swelling, progressively increasing in size since birth. Computed tomography of the pelvis and lower limbs confirmed the diagnosis of extensive VM of pelvis and perineum. She underwent selective angiogram which revealed venous malformations of left vulva and gluteal region with no arterial supply to the lesion. Surgical excision was performed but complicated with bleeding which necessitate multimodal hemostatic procedures and blood transfusion. There is no recurrent swelling after 5 years follow up. Surgical excision of vulvar venous malformation offer good cosmetic outcome. However, appropriate planning with axial scan, angiogram and operative technique including multimodal hemostatic measure are important steps to achieve good result with no recurrence. We discussed on challenges on management of this case in relation to the currently available literature.
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