Background: Deep Aggressive Angiomyxoma (DAA) is a rare benign tumor with about 250 cases reported worldwide. It was first reported in 1983. This tumor is commonly found in women of reproductive age, with the highest incidence in the 3rd and 4th decades of life. DAA is most often found in the pelvic, genital, inguinal region, or perineum. Case Report: Nulligravida woman 52 years old, with enlargement of the vulva that had felt since ± 20 years ago. A mass was found on local examination of the right vulva with a size of ±50x40x10cm, which was supple, mobile, and with no pain. Enlarged inguinal lymphadenopathy was also observed. MRI showed a well-defined lesion at the right vulvar region (which visualized the size of ± AP 14.95 x LL 31.80 x CC 15. 3 cm) with the signal intensity hypointense on T1WI, hyperintense on T2WI and FatSat, restricted on DWI, with no enhancement was seen. A wide excision was conducted, and post-excision measurements showed a right vulvar mass size was 50x42x10cm, with 14.8kg in weight. Conclusion: DAA is a rare benign tumor that can disturb patients. The nature of the tumor should be evaluated carefully using radiological and pathological approaches. A wide excision can remove the tumor completely, but control and follow-up are mandatory to evaluate the completeness of the procedure and monitor for any relapse.