Vulvar cancer represents 1% of cancers in women; vulvar neuroendocrine carcinoma is extremely rare, with less than 20 published cases. Its behavior is very aggressive, producing early local recurrences, lymph node and distant metastases. The case of a 60-year-old patient who consulted for a vulvar lesion of 6 months of evolution, with extension to the lower third of the urethra and vagina and left inguinal nodal plastron is described. The vulvar biopsy reported malignant round cell neoplasm and the immunohistochemistry result was compatible with neuroendocrine carcinoma. She received concurrent chemotherapy and radiotherapy, evidencing a complete response in the vulva and a partial response in the inguinal region.Later, bilateral inguinofemoral lymphadenectomy was performed. Neuroendocrine carcinoma of the vulva should be considered when managing malignant vulvar pathology, timely diagnosis requires histology and immunohistochemistry to establish an adequate prognosis and treatment.