Introduction/Background Angiomyofibroblastoma is part of the benign mesenchymal tumors of the genital tract and was first described by Fletcher in 1992. The vulva represents the main presentation site, but cases have been described at the level of the vagina, cervix, fallopian tubes, the scrotum and the ischiorectal fossa. It presents as a mass, generally painless, with well-defined edges and without compromising the overlying skin. It is normally less than 5 cm, but cases of up to 37 cm have been described.

Methodology A 45-year-old woman with a 1 month history of painful vulvar mass of progressive growth. Physical examination reveals a stony, mobile mass of 5× 5cm in right labia majora. Initial immunostaining suggested an AMFB, so she was taken to vulvectomy. A review was made of all articles in English or Spanish, published until March 10, 2019, related to the diagnosis and treatment of vulvar AMFB. The search included the PubMed, Embase, Cochrane, LILACS and Scielo databases, with the keywords ”Vulvar Angiomyofibroblastoma” OR ”Angiomyofibroblastoma of the vulva with sarcomatous transformation”.

Results A total of 69 reported cases were found. In our knowledge, the case presented is the first report of AMFB in Colombia. The age of the patients ranged between 16 and 82 years, with an average of 43 years. The main presenting symptom was the presence of a non-painful vulvar mass, although some of the patients in the reported cases reported pain and dyspareunia (8/69). The evolution time varied between 1 month and 9 years with an average of 21 months, and the size between 1 and 37 centimeters with an average of 7 centimeters. Of the cases taken to immunohistochemical study, 86% were positive for estrogen receptors and 80% for progesterone receptors, 88% were positive for desmin and 98% for vimentin, 92% were negative for S100 and the cases that were positive corresponded to the lipomatous variant, 43% were positive for α-SMA and 34% for CD 34. Definitive diagnosis is based on histological findings. In this reviewed cases, only one relapse was reported. The universal treatment is excision and only one case of relapse was reported, which corresponded to the only reported case of malignant transformation.

Conclusion Vulvar AMFB is a rare mesenchymal tumor, with exclusive surgical treatment and excellent prognosis, although it may have malignant transformation. The reported case could represent the second case of AMFB with malignant transformation.