本文报道一例63岁男性患者，因“右上腹部隐痛2个月”就诊。经影像学与病理学检查，确诊为同时性肝细胞癌（CNLC Ⅰa期）及十二指肠乳头癌（中-低分化腺癌伴印戒细胞癌成分）。患者既往有慢性乙型肝炎病史。经MDT讨论，行腹腔镜下肝S5段局部切除联合胰十二指肠切除术，实现R0切除。术后2个月发生肝转移，TACE、替雷利珠单抗免疫治疗及FOLFOX4方案化疗后达完全缓解，术后生存期已超过16个月。本例同时性肝与十二指肠乳头双原发癌极为罕见，且临床易漏诊，提示在确诊一种恶性肿瘤后，仍需进行全面评估以排除其他原发性病灶，PET-CT 在此类多原发癌的早期识别中展现出重要价值。通过个体化综合治疗与全程管理的多模式策略，该病例最终实现了良好的肿瘤控制效果，为类似罕见情形的临床诊治提供了参考。

In this report, a 63-year-old male patient was admitted due to "dull pain in the right upper abdomen for 2 months". Imaging and pathological examination confirmed the diagnosis of synchronous hepatocellular carcinoma (CNLC Ⅰa stage) and duodenal papillary carcinoma (moderately-poorly differentiated adenocarcinoma complicated with signet ring cell carcinoma). The patient had a history of chronic hepatitis B. After Multidisciplinary team (MDT) consultation, laparoscopic local resection of segment 5 of the liver combined with pancreatoduodenectomy were performed to achieve R0 resection. Liver metastasis occurred 2 months after operation, and it was completely relieved after TACE, tirelizumab immunotherapy and FOLFOX4 chemotherapy. Postoperative survival time has exceeded 16 months. This case of synchronous double primary carcinoma of the liver and duodenal papilla is extremely rare, and it is likely to miss the diagnosis in clinical practice, suggesting that after the diagnosis of one type of malignant tumor, comprehensive evaluation is still needed to exclude other primary lesions. PET-CT shows important value in the early identification of such multiple primary cancers. Through multi-mode strategy of individualized comprehensive treatment and whole-course management, this patient achieved favorable tumor control effect, providing reference for clinical diagnosis and treatment of such rare cases.