Background: Synchronous double primary cancer consisting of hepatocellular carcinoma (HCC) and appendiceal mucinous adenocarcinomas has not been reported in the world so far. Liver metastasis of digestive tract tumor is so common that it is easy to ignore the existence of double primary cancer in the digestive system. Appendiceal malignant tumor itself is easy to be misdiagnosed or missed. For the better understanding of HCC and appendiceal mucinous adenocarcinomas, the information of our recent case has been summarized.Case presentation: A 58-year-old man was admitted with repeated upper abdominal pain for one week. Imaging examination and liver biopsy pathology were diagnosed as hepatocellular carcinoma. Colonoscopy showed no obvious organic lesions in the terminal ileum and the whole large intestine. We had anatomical resection of S5, S6 segment of liver. At the same time, two suspicious lesions in the right liver were ablated by microwave. But at the end of the operation, we accidentally found a hard mass about 3cm x 3cm in size at the end of the appendix. And the rapid pathological results showed appendiceal adenocarcinoma, so we had a right hemicolectomy. So far, the diagnosis of the patient was changed to synchronous double primary cancer consisting of HCC and appendiceal mucinous adenocarcinomas.Conclusions: It is the first time we report a case of synchronous double primary hepatic cancer consisting of HCC and appendiceal mucinous adenocarcinomas in the world. Due to the rarity and unspecific clinical features, it is extremely challenging to be diagnosed preoperatively. It requires us to have a more comprehensive understanding of the patient's medical history, careful physical examination and comprehensive exploration of the abdominal cavity during the operation, so as to avoid missed diagnosis. Patients with HCC and appendiceal mucinous adenocarcinomas are generally associated with poor prognosis. It needs we put forward new countermeasures for the new question.