Objective To explore the clinical features, treatment methods and prognostic factors of primary appendiceal tumors. Methods The clinicopathologic data of 82 patients with primary appendiceal tumor who met the inclusion and exclusion criteria and were treated in the Affiliated Hospital of Southwest Medical University from January 2013 to November 2022 were retrospectively collected, and the clinicopathologic characteristics of the patients were summarized. Log-rank and Cox proportional hazard regression models were further used for univariate and multivariate survival analysis for patients without other malignant tumors. Results Among the 82 patients with primary appendiceal tumor, 9 cases (11.0%) were complicated with other malignant tumors. Low-grade mucinous tumors were the most common (51.2%, 42/82), 25 cases were complicated with appendicitis and 12 cases were complicated with intestinal obstruction. Common clinical manifestations included abdominal pain, changes in bowel habits, abdominal distension, vomiting, nausea, etc. Among the patients without other malignancies, 69 patients were followed up for a median of 32 months (4–106 months), 18 of whom died. The 1-year, 2-year, 3-year and 5-year cumulative survival rates were 91.1%, 87.5%, 80.5% and 60.7%, respectively. Multivariate Cox proportional hazard regression analysis showed that platelet-to-lymphocyte ratio (P=0.004), anemia (P=0.015), tumor metastasis (P=0.007) and diabetes mellitus (P=0.011) were prognostic factors. Among them, patients with platelet-to-lymphocyte ratio >190.1, anemia, tumor metastasis and diabetes mellitus had poor prognosis. Conclusions The main pathological type of primary appendiceal tumors is low grade mucinous tumors. It lacks characteristic clinical manifestations, with abdominal pain and changes in bowel habits as the main manifestations, and can be accompanied by appendicitis and intestinal obstruction. There is no unified standard for treatment. For patients characterized by prognostic risk factors including platelet-to-lymphocyte ratio >190.1, anemia, tumor metastasis and diabetes, further monitoring and intervention are required.