There are many pathological types of pancreatic space-occupying lesions with different clinical manifestations, among which pancreatic ductal adenocarcinoma can be found usually and is high malignant.In addition to pancreatic cancer, some rare pancreatic tumors appear to be a benign process, and the patients′ prognosis is totally different.But it is tough to distinguish from pancreatic cancer.The combination of clinical manifestations, laboratory examination and imaging examination has great clinical significance for timely and accurate diagnosis, treatment and prognosis evaluation.Herein we present a review of rare pancreatic space-occupying lesions with regard to the clinical characteristics that hopefully will contribute to better recognition and differential diagnosis in the near future.