A literature review dealing with epidemiological and etiologic aspects of squamous cell carcinoma of the vulva is presented.

It is a rare disease; the incidence is 1-2 per 100 000 women per year.

Among women more than 75 years of age, the incidence is at least ten times higher.

The cause of vulvar carcinoma is not known.

In contrast to what has been stated for many years, in recent case-control studies no evidence was found that diabetes mellitus, obesity, vascular diseases, nulliparity, an early menopause and syphilis are risk factors for developing a vulvar carcinoma.

The factors that play a role in the etiology of vulvar carcinoma are: vulvar dystrophy, a history of genital HPV-infection, a history of cervical neoplasia, advanced age, smoking and a compromised immunity.