Neuroendocrine small cell carcinoma of the vulva is a rare and aggressive cancer with a pejorative behavior. Because it is rare, there is no consensus to date. We report the fourteenth case of a 52-year-old woman with neuroendocrine small cell carcinoma of the vulva, treated by surgery and combined chemoradiotherapy 46 Gy and cisplatinum-étoposide and adjuvant chemotherapy. Twelve months after the diagnosis, she is alive with no evidence of disease recurrence. A review of the literature on this topic is also presented.