A case of localized small cell neuroendocrine carcinoma of the vagina is reported. The patient was treated with concurrent chemoradiation with significant toxicity but obtained a complete response. Thirteen months after therapy the patient developed distant metastasis and died shortly thereafter. Review of the literature found that patients treated with local therapy alone had a shorter survival and died of systemic disease. As with small cell neuroendocrine carcinomas arising from other sites, systemic relapse remains an important issue that warrants combination therapies, although in pelvic sites this may be associated with an increase in side-effects.