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BACKGROUND Mesonephric adenocarcinoma (MNA) of the female reproductive system is a rare tumor arising from remnants of the mesonephric duct, which is mainly located in the cervix. MNA often occurs in adult women. Due to the rarity of the disease and few reports, the specific clinical features have not been established. CASE SUMMARY We present a case of a cervical MNA in a 48-year-old woman with an incidental intra-operative diagnosis who received postoperative chemotherapy. Rare lung metastases were detected during follow-up. The existing literature is reviewed. CONCLUSION The clinical manifestations, pathological characteristics, diagnosis, treatment, and prognosis of MNA have been summarized through the review of the existing literature and the case in this paper. Due to the rarity of this disease, it is very important for the research of MNA in the future.