Introduction/Background The objective is to report a case of a small cell neuroendocrine carcinoma (SCNC) of the vagina, diagnosed and treated at the Hospital Universitario San Ignacio in Bogotá, Colombia.

Methodology The patient is a 60 year old female with a 10 months history of a painful mass in the vagina. At physical examination with a friable 4 cm red mass in the lateral and posterior wall of the vagina that invaded the paravaginal tissue. Imaging showed bilateral pelvic lymph nodes of metastatic appearance that were biopsied with final morphological and immunohistochemical pathology report consistent with SCNC of the vagina. A systematic review of the literature of similar reported cases was conducted to identify articles that examined the diagnosis and treatment of vaginal SCNC published in english and spanish up to March 31, 2022. The following electronic databases were searched: PubMed, Embase, Cochrane, LILACS and Scielo. The following search terms were used: ‘neuroendocrine carcinoma’ or ‘small cell carcinoma’ and ‘vaginal neoplasms’.

Results We identified 37 publications that included a total of 46 cases. Our case represents the first case report of a vaginal SCNC in our country.

Conclusion SCNC is rare. There is no standard of treatment. It has a poor prognosis and mortality rates in the first year after diagnosis are as high as 85%. Multicentric studies are necessary to identify prognostic factors and establish treatment strategies.