Vulvar and vaginal cancers are rare and account for approximately 7% of cancers of the female reproductive tract. Vulvar and vaginal neoplasia share similar risk factors: human papillomavirus infection, previous cervical intraepithelial neoplasia or cervical cancer, current smoking, sexual factors, and immunosuppression. Several treatment options are available for patients with documented histologic high-grade intraepithelial vulvar or vaginal neoplasia, including excision, laser vaporization, and 5-fluorouracil. After treatment, lifetime follow-up with cytology and colposcopy is recommended. With the widespread use of the human papillomavirus vaccine, one half to two thirds of vulvar and vaginal cancers may be prevented. Patient education regarding reduction of risk factors for progression and close surveillance of at-risk individuals may prevent the progression to invasive disease.