OBJECTIVES: Pancreatic cancer is considered one of the toughest cancers to treat, with extremely poor prognosis.  The objective of this research was to conduct a systematic review of epidemiology and the burden of pancreatic cancer. METHODS: A systematic literature search for epidemiology and the burden of disease studies was undertaken for the databases Pubmed, Embase, Biosis, Google Scholar and Cochrane. Data was collected for the study type, methods, country and key findings. Extracted study data included pancreatic cancer incidence, complications, mortality, available treatment options, as well as healthcare resource utilization and medical costs associated with pancreatic cancer. Critical analyses of study quality and data gaps were analyzed at the country level. RESULTS: A total of 328 studies were identified based on the keywords. Of these, 32 studies met the inclusion criteria. Studies indicate that pancreatic cancer has an extremely poor prognosis: for all stages combined, the 1- and 5-year relative survival rates are 25% and 6%, respectively. Pancreatic cancer is the fourth most common cause of cancer-related deaths in the United States and the eighth worldwide. More than 50% of patients come to clinical attention with metastatic disease, and an additional 30%–40% present with locally advanced tumors. Current treatments include surgery and palliative chemotherapy such as gemicitabine and gemcitabine/erlotinib combination. Recently nab-paxclitacel was approved based on a 1.8 month improvement in the overall survival.  CONCLUSIONS: This systematic review shows that patients with pancreatic cancer have a very low survival rate. There is an urgent need for new treatments for these patients.