【Abstract】Objective This research was aimed to determinate the clinical and image data from the patients being diagnosed with primary appendiceal carcinoma and provide novel strategy with diagnosis. Methods The study of retrospective analysis investigate 3286 appendectomy data in the Yichang Central People’s Hospital from May 2012 to June 2016. 26 primary appendiceal carcinoma patients were confirmed by histopathological examination. The research was focused on the clinical presentations, laboratory examination and image features and the characteristics. ResultsmThe patients included 14 males and 12 females, the average age was 56.7±16.8. The common presentations were abdominal pain, abdominal mass and nausea. CEA and CA199 were easily to be forgotten before operation. The sensitivity of CEA and CA199 were 33.3% and 53.3%, respectively. Ultrasonography of the abdomen shown mixed echoic lesions. The characteristics of abdominal CT were enlarged appendix, thickened wall, effusion and increased lymph nodes. Colonoscopy may discover cauliflower mass and luminal narrowing. Conclusion The primary appendix carcinoma lacks specific clinical findings. There were no obvious findings in the abdomen CT. Colonoscopy and tumor markers may be ignorant. their sensitivity is also limitation. Therefore, it should be combined with CEA, CA 199 and colonoscopy and other examinations to make correct diagnosis quickly for appendiceal lesions in the CT or USG.