Abstract： Objective To improve the diagnostic and therapeutic efficacy for young patients with malignant tumors of pancreas at the early stage, and describe the clinical characteristics of this group of patients. Methods The clinical data of 46 young patients less than forty years old with malignant tumors of pancreas received treatment and follow up, including clinical features, clinicopathological parameters, status of misdiagnosis, CA 19-9, CEA, were retrospectively reviewed. Results The main manifestations of young patients with malignant tumors of pancreas were abdominal bloating (78.26% ), epigastric discomfort (73.91%), weight loss (73.91%), loss of appetite (69.57%), abdominal pain (60.87%). Majority of the tumors were located in pancreatic head. 30 cases (65.22%) had pathological evidence of metastasis, among them 13 cases had multi-organ involvement. Pancreatic exocrine tumor accounted for 72.73% (24/33) of all the tumors, while endocrine tumor accounted for 27.27% (9/33). The positive rates of CA 19-9 and CEA were 73.91% (34/46) and 32.6% (15/46). The misdiagnosis rate of young patients with malignant tumors of pancreas was up to 80.43% (37/46). The main misdiagnoses were acute or chronic gastritis (36.96%). The rate of radical operation for young patients with malignant tumors of pancreas was 23.91%, and the median survival was 12.6 months. Conclusions The symptoms of young patients with malignant pancreatic tumors are non-speciflc and the misdiagnosis rate was high and the prognosis was poor, therefore, the early diagnosis of pancreatic malignant tumors shall be emphasized.