Vulvar intraepithelial neoplasia (VIN) and human papilloma virus (HPV) are the main pathways for development of vulvar carcinoma in young women. While, non-HPV associated pathway is the usual pattern seen in postmenopausal women. Herein, the authors report a case of 26-year-old lady with keratinizing vulvar squamous cell carcinoma (SCC), in which vulvar intraepithelial neoplasia (VIN) of the simplex type and p53 expression were absent. Moreover, no high risk HPV was detected by the highly sensitive real time-polymerase chain reaction (RT-PCR) technology on the paraffin embedded tumor tissue. Therefore, in addition to the patient's young age, she had morphologic and molecular patterns that are usually seen in elderly women. The authors believe that this is an unusual presentation in such a very young lady. The cause of this cancer in this case is not completely understood and indicates the necessity to sample any suspicious lesions at this site at any age.