Pancreatic cancer, consisting mostly of invasive pancreatic ductal adenocarcinoma (PDAC), arises from the ductal cells of the exocrine pancreas and is the 4th leading cause of cancer-related deaths in the United States. Pancreatic cancer has the highest mortality rate among the major cancers, with a 5-year survival rate of only 8%–10%. Clinical presentation includes symptoms of abdominal pain, jaundice, and weight loss. Diagnosis is made by CT, MRI, and endoscopic ultrasonography (EUS). Management by surgical resection, usually with neoadjuvant or adjuvant chemotherapy, provides the only chance for cure in the 15%–20% of patients who have resectable disease at the time of diagnosis. Other rare malignant tumors arising from the exocrine pancreas are acinar cell carcinoma and pancreatoblastoma.