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Primary carcinoma of the Bartholin's gland (BG) is a rare malignancy. There are extremely rare cases of small cell neuroendocrine carcinoma (SCNC) of the BG reported in the English literature. A postmenopausal female presented with a 1‑month history of increasing pain and swelling on the left vulva consistent with spontaneously bleeding. Pathology identified SCNC that arose in BG. The patient was treated with a radical wide local excision and bilateral inguinal lymph node dissection followed by six courses of chemotherapy. One month after primary treatment, without any pelvic recurrence or abnormal tumor markers indications, distant metastasis of the liver was diagnosed and VI hepatic lobectomy was performed. The patient maintained regular adjuvant chemotherapy every month under outpatient surveillance and has no local recurrence or distant metastasis. [ABSTRACT FROM AUTHOR]