Introduction: Primary vaginal malignant tumors are rare, especiallyneuroendocrine tumors. The primary small cell carcinoma of the vagina is anaggressive tumor with poor prognosis because of early recurrence andmetastasis. Aim: to report the complexity of treatment of primaryneuroendocrine carcinoma of vagina, and its metastasis. Material &methods: we report a retrospective case of primaryneuroendocrine carcinoma of vagina treated in our institution. Results: We report a case of a37-year-old woman who was diagnosed with a small cell carcinoma of the vaginawithout distant metastasis. The primary vaginal tumor was treated by surgery,brachytherapy, and chemotherapy. The first recurrence appeared seven years afterinitial diagnosis, which was a pulmonary metastasis, treated surgically. Sevenyears after this recurrence, she was treated with chemotherapy and surgery foranother pulmonary metastasis. Fifteen years after the initial diagnosis, thepatient underwent surgery for brainmetastasis. She died eighteen months after the third recurrence.Conclusions: This case splendidly illustrates a longsurvival term of a tumor known with poor prognosis.