Peutz-jeghers syndrome (PJS) is an autosomal dominant genetic disease whose pathogenesis is mainly the inactivation of STK11/ LKB1 gene. Its clinical features include multiple polyps of the gastrointestinal tract, abnormal pigmentation of skin and mucous membrane, and significantly increased risk of various malignant tumors Several diseases of the female reproductive system have been associated with PJS, including annular tubule neoplasms of the ovary, microdeviated adenocarcinoma of the cervix. However, cervical adenocarcinoma combined with small cell neuroendocrine carcinoma has rarely been reported. Here, we report a case of Peutz-Jeghers syndrome manifested by cervical adenocarcinoma and small cell neuroendocrine carcinoma and combined with STK11/LKB1 gene germline mutation, aiming to help us understand this rare disease and call for multidisciplinary attention to Peutz-jeghers syndrome.