Introduction and importanceBasal cell carcinoma (BCC), is the most prevalent skin cancer with favorable prognosis, and lymphatic or hematogenous metastases are quite rare. Here we report an uncommon case of vulvar BCC.Case presentationA 68-year-old woman with 5-year history of an asymptomatic lump on the right side of the vulva. The patient complained of progressive itching, pain, and color changes to brown for 3 months before visiting the clinic. Excisional biopsy revealed the lesion to be a BCC. After thorough negative metastasis work up the patient underwent a bilateral inguinofemoral lymphadenectomy and a wide local excision with a one-centimeter margin and final histology report confirmed the diagnosis of BCC. (HPV DNA and viral markers were negative, the bimanual exam was normal, both colposcopy and vaginoscopy were normal. There was no acetowhite lesion).Clinical discussionTo avoid delayed diagnosis, any persistent lesion even asymptomatic, in the vulvar region, especially when pigmented, irritating, or expanding in size, should be biopsied and investigated histologically, regardless of the patient's age.ConclusionAny persistent lesion in the vulvar region, especially when pigmented, irritating, or expanding in size, should be biopsied and investigated histologically, regardless of the patient's age.