Vulvar carcinoma is a rare female genital neoplasm. Although numerous molecular defects in vulvar carcinomas have been reported until now, no molecular markers that could be applied in daily clinical work have been identified so far. However, there is emerging evidence that specific mutations and gene expression patterns may be used as diagnostic tools in oncology. In this article we systematically review genetic alterations that may contribute to the development and progression of vulvar carcinoma. We conclude by suggesting molecular markers of potential clinical value in the diagnostics of this type of cancer.