Rare case of huge vulvar angiomyofibroblastoma in a young female

•Huge angiomyofibroblastoma of the Vulva.•Differential diagnosis between vulvar angiomyofibroblastoma and vulvar aggressive angiomyxoma.•Comprehensive approach diagnosing and therapy of vulvar angiomyofibroblastoma. Mesenchymal tumours of the vulva are rare and consist of two types, difficult to distinguish but with different prognoses. Angiomyofibroblastoma (AMFB) is a benign tumour, whereas Aggressive Angiomyxoma (AA) is an infiltrating tumour. We describe a 22-year-old nulliparous patient with a vulvar mass sized 19 cm in diameter. After preoperative assessment by ultrasound, chest X-ray, and MRI, wide excision on the tumour was done and diagnosed as AMFB. Differentiation from AA is being discussed.