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Objective To investigate the clinical features, pathological features, differential diagnosis, and prognosis of solid pseudopapillary neoplasm (SPN) of the pancreas. Methods A retrospective analysis was performed for the clinical data of 14 patients with SPN who were treated in our hospital from January 2014 to February 2018, and related articles were reviewed. Results There were 11 female and 3 male patients with an age of onset of 13-77 years (mean 33.1 years). Most of them attended the hospital due to lesions found by physical examination or the presence of upper abdominal pain. Radiological examination revealed space-occupying lesion in the pancreas. The maximum diameter of the tumor ranged from 0.6 cm to 22 cm. Histological examination showed that most tumors were composed of solid areas and pseudopapillary areas, with a microcystic structure in local lesion. Immunohistochemistry showed positive Vimentin and negative CgA ,Glucagon, Gastrin, and Insulin in all patients. Some patients were positive for β-catenin (13/14), CD10 (10/14), CD56 (9/14), Syn (8/14), AA-T (11/14), PR (8/14), CyclinD1 (9/14), CA19-9 (3/14), CK-pan (9/14), CEA (1/14), and P53 (1/14). Ki-67 index was ≤10% in all 14 patients. Conclusion SPN of the pancreas should be diagnosed with reference to clinical data, imaging examination, histological features, and immunohistochemistry. The microcystic structure has a certain value in the diagnosis of SPN. [ABSTRACT FROM AUTHOR]