Context Solid pseudopapillary neoplasms of the pancreas are mainly found in females and account for <2% of pancreatic tumors. They have nonspecific clinical presentation with vague radiologic features and are often histologically benign. Despite the uncertain histogenesis and the low grade of malignancy, these neoplasms present a select panel of immunostains which advantage pathologists to differentiate from other tumors of the pancreas. The current study aims to present the treatment of 2 cases of solid-pseudopapillary neoplasm of the pancreas in our hospital and a literature review on the topic. Case report Both patients were females with a mean tumor size of 5 cm. Preoperative diagnosis was based on distinctive features of the neoplasm in fine needle aspiration cytology in one patient. The two procedures performed were enucleation of the tumor and a distal pancreaticosplenectomy. Both patients are on a regular follow up and no recurrence has been detected 2 years after surgery. Conclusions It is important to differentiate solid pseudopapillary neoplasms from other pancreatic neoplasms because of the low potential for malignancy and a favorable prognosis. Fine needle aspiration cytology is the most valuable tool for diagnosis and surgical planning. Surgery is the primary therapeutic modality and in patients with distant metastasis or adjacent organ invasion aggressive approach should be considered. Local resection or enucleation can be performed for small tumors in selected cases. There are limited data about chemotherapy and radiotherapy modalities due to the availability of limited case series or reports only. Despite the excellent prognosis, the inability to predict malignant behavior mandates a long-term follow-up post-surgery.