Vulvar cancer is an uncommon gynecological malignancy primarily affecting postmenopausal women. No specific screening exists and the most effective strategy to reduce the incidence of vulvar cancer is the opportune treatment of predisposing and preneoplastic lesions associated with its development. Although vulvar cancer may be asymptomatic, most women present with vulvar pruritus or pain or have noticed a lump or ulcer. Therefore, any suspicious vulvar lesion should be biopsied to exclude invasion. Once established, the most common subtype is squamous cell carcinoma. The treatment of vulvar cancer depends primarily on histology, clinical, radiological, or surgical staging. Treatment is predominantly surgical and/or adjuvant radiation therapy, particularly for squamous cell carcinoma, although definitive concurrent chemoradiation is an effective alternative, particularly for advanced tumors. In those cases where vulvar cancer presents with distant metastases, systemic therapy is modeled after cervical cancer, a more common cancer where there is more robust data to guide treatment. Management should be individualized and carried out by a multidisciplinary team in a cancer center experienced in the treatment of these tumors.