Background: Aggressive angiomyxomas (AAM) are rare mesenchymal neoplasms that occur more commonly in women. The mainstay of treatment is surgical excision which can be performed via various approaches. However, there are limited reports on minimally invasive surgeries in the treatment of AAM.We report a successful case of single-port laparoscopic and transvaginal resection of a pelvic AAM.Case Description: Our patient was a 45-year-old female who presented with symptomatic anaemia from menorrhagia and a vaginal lump of one year's duration that was worsening and affecting her quality of life (QOL). There was a 20 cm prolapsed, reducible smooth mass from the vagina on examination. An magnetic resonance imaging (MRI) scan showed a 23.9 cm × 12.7 cm × 8.4 cm extraperitoneal, mildly enhancing soft tissue mass sited between the uterus, vagina anteriorly and the rectum posteriorly, with a significant prolapsed component which showed no overt infiltration or invasion into the adjacent structures or sacrococcygeal spine. She underwent a single-port laparoscopic total hysterectomy, bilateral salpingectomies, and resection of the retroperitoneal mass. Intra-operatively, the uterus was 12 weeks in size. There was a large retroperitoneal mass on the right side, of which 40 cm had prolapsed through the vagina. The mass extended to the right common iliac vessels and down to the Pouch of Douglas. It was adherent to the pelvic sidewall, ureters, and retroperitoneal space. There was no direct involvement of the uterus, bowels, or ureters. The patient recovered well postoperatively, and the histology of the mass was an AAM that was strongly positive for oestrogen and progesterone receptors. To date, the prognosis was good as the woman was well with no recurrence for two and a half years. She regained her QOL. She will need to continue her follow-up to assess the long-term prognosis.Conclusions: A minimally invasive approach is increasingly used in various oncological surgeries, especially gynaecology oncological cases. Our case demonstrates that a combination of single-port laparoscopic and transvaginal techniques can be performed with the benefits of complete enucleation and resection of the tumour, less scarring and lesser postoperative pain. This study provides a possible alternative surgical procedure for patients with AAM that experienced surgeons can perform safely.