Squamous cell carcinoma of the vulva is a rare disease, mainly seen in elderly women. Risk factors are advanced age, an immunocompromised status, longstanding vulvar dystrophy, VIN, a history of vulvar human papillomavirus infection, and a history of cervical cancer. Vulvar cancer should be considered as a skin tumor and detection is possible in an early stage. However, because of patients' and doctors' delay, one in three vulvar cancers is not treated before an advanced stage. The tumor metastasizes mainly lymphatogenic. Spread starts in the inguinal lymph nodes. In the middle of this century, standard treatment, consisting of an en bloc dissection of the vulva and inguinal lymph nodes has been developed and applied. As a result, considerably improved survival rates were achieved: up to 90% 5-year survival rates for patients without lymph node metastases. However, complication rates were high. In recent years, a more individualized approach has replaced standard treatment. Surgical treatment now depends on the localization, size and extent of the tumor, and is followed or preceded by radiotherapy in selected cases. The role of chemotherapy in advanced disease is currently being studied in several referral centers. The most important success in the treatment of vulvar cancer in recent years is the maintenance of high survival rates despite considerably less extensive surgical treatment, resulting in lower complications rates. An important challenge for the near future will be the improvement of the management of advanced disease. However, an even more difficult issue may be the prevention of such large lesions. The reduction of treatment delays requires a considerable effort in education of both health care workers and the general public.