BACKGROUND Verrucous carcinoma (VC) of the vulva is a variation of squamous carcinoma (SCC). Etiology and treatment of VC are still unclear. CASE REPORT A 50-year-old female visited our clinic with a giant vulvar tumor (8 cm of diameter maximum). Biopsy revealed a suspicious well differentiation squamous cancer. PET/CT (positron emission tomography/computed tomography) scan found suspicious lymph node in bilateral iliac vessel region and bilateral inguinal region. She underwent radical vulvectomy and bilateral inguinal lymph node dissection, and bilateral pelvic lymph node dissection. Pathology turns out to be VC and no lymph nodes involvement. Due to the large defection, vulvar reconstruction was performed 5 weeks later using skin grafts and pudendal thigh flap. This patient was disease free after 12 months follow-up. CONCLUSIONS In patients with VC, a satisfactory biopsy is important and systemic inguinal lymphadenectomy might be omitted. For patients with large defection, flap-based reconstruction is recommended.