Context Solid-cystic pseudopapillary tumor of the pancreas is a rare tumor, but has favorable prognosis even in the presence of distant metastases. It is often associated with diagnostic and therapeutic challenges. Case reports Described are two cases of solidcystic pseudopapillary tumor of the pancreas managed in our hospital between January 2000 and January 2010. One female and one male with mean age of 42 years (range: 33 to 51 years). Data collected were: presenting symptoms, signs, imaging and management. Literature review was obtained from computer generated MEDLINE and Google Scholar search for solid-cystic pseudopapillary tumor of the pancreas between 1980 and 2010. Articles relevant to the scope of this paper were selected. The incidence of solidcystic pseudopapillary tumors of the pancreas in our institution is 2 cases in 10 years; i.e., 0.2 cases/year. Clinical presentation include; palpable abdominal mass, vague abdominal discomfort, increasing abdominal girth, significant weight loss, nausea and vomiting in both patients. The mean diameter of the tumors was 7.0 cm (range: 5.9 to 8.0 cm). Pancreaticoduodenectomy was performed on both patients. None received adjuvant therapy and no cancer recurrence was detected on follow-up. Conclusion Solidcystic pseudopapillary tumor of the pancreas is a rare tumor with a favorable prognosis even with distant metastases; therefore, attempts must be made to differentiate it from other pancreatic neoplasms. Clinical and radiological studies alone are insufficient to establish the diagnosis of a pancreatic mass and tissue biopsy should be sought. Surgery is generally curative and should be pursued independent of size and distant metastases.