The incidence of vulvar squamous cell carcinoma (SCC), the most common vulvar cancer in women, is increasing worldwide. Furthermore, despite the increasing prevalence, this disease is commonly misdiagnosed by physicians. Unfortunately, late-stage vulvar SCC has a poorer prognosis compared with early vulvar neoplasia and requires aggressive therapy such as radical surgical intervention. Therefore, early diagnosis by physicians is critical to reduce both the mortality and morbidity rates of vulvar SCC. This review discusses the etiology, clinical characteristics, and diagnostic staging of vulvar SCC. Treatment options are reviewed, including novel topical immunotherapies such as imiquimod.