Vulvar cancer is an uncommon disease representing 3–5% of female genital tract malignancies. Squamous cell cancer accounts for approximately 90% of all primary vulvar malignancies. The incidence of in situ cancer has almost doubled over the past 30 years. The overall rate of invasive squamous cell cancer has remained relatively stable. Radical vulvectomy and en bloc groin dissection with pelvic lymphadenectomy have been considered standard treatment for all patients who have operable disease. In locally advanced vulvar cancer, partial resection of the urethra, vagina, anus or exenteration when necessary, has also been applied with severe post-surgical complications, poor quality of life and unsatisfactory survival rates. Chemoradiation followed by tailored surgery represents an attractive therapeutic option for advanced cases, to avoid ultraradical surgery and improve patient outcome. Chemotherapy has also been used in neoadjuvant therapy and in palliative treatment of patients with distant metastases. Numerous series have reported on the use of external beam radiation, often with concomitant chemotherapy, to shrink the primary tumor. Chemoradiation facilitates more limited resection of the tumor bed and lymphadenectomy on an individual basis, and initial response rates are approximately 90%. With the experience now accrued, preoperative chemotherapy with radiation should be regarded as the first-choice treatment for patients with advanced vulvar cancer.