Background: Vulvar carcinoma is rare, 4% of gynecological cancer worldwide and basal cell carcinoma of these represents 2 to 3%; Its diagnosis is delayed due to carelessness of the patient and doctor.
Objective: To present a clinical case of basal cell carcinoma of the vulva and review of the literature.
Clinical case: A 58-year-old patient, without comorbidities or risk factors, who presented a nodule in the upper third of the right labia majora; Excisional biopsy was performed with margins free of macroscopic tumor, basal cell carcinoma was reported.
Methodology: A narrative review is carried out from 2014 to 2021 in PubMed, scholaris, SciELO Google scholar and books, finding 27 articles and only 20 were included for reliability, impact factor.
Results: Vulvar cancer represents 4% of gynecological cancer worldwide. 0.4% of all basal cell carcinomas occur in the genital region, and in the vulva it represents 2 to 4% of all vulvar cancers. Basal cell carcinoma of the vulva most commonly presents as a solitary pink or pigmented papule or plaque on the labia majora; bilateral and multifocal lesions may occur. Basal cell carcinoma of the vulva is an extremely rare tumor that rarely metastasizes or spreads. . Primary treatment should consist of wide local excision and continuous monitoring.
Conclusion: Any suspicious lesion of the vulva must be biopsied to reach a timely diagnosis and treatment, the rarity of these lesions and the lack of care on the part of the patient and the negligence of the doctor not to perform an examination of the vulva, the lesions progress until symptoms occur or are discovered during medical examination, vulvar cancer is suspected, radical excisional biopsy is the correct therapeutic approach.