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Context: Acinar cell carcinoma of the pancreas is a rare malignancy that may have acinar and endocrine differentiation. Clinical practice guidelines exist for pancreatic ductal adenocarcinoma. However, treatment protocols for acinar cell carcinoma of the pancreas have not been standardized., Case Report: We describe a case of a 44-year-old woman presenting with low grade fever and mid-abdominal tenderness secondary to a pancreatic mass with acinar and endocrine differentiation metastatic to the liver. The patient received multiple lines of conventional and investigational chemotherapy regimens. This included cycles of gemcitabine, irinotecan, oxaliplatin, docetaxel, capecitabine, octreotide, leucovorin, 5-fluorouracil, cisplatin, protein kinase C inhibitor, tyrosine kinase inhibitor, and a novel taxane. The patient developed Clostridium difficile colitis and septic shock resulting in death 37 months after the diagnosis of acinar cell carcinoma of the pancreas., Conclusion: This is a case of acinar cell carcinoma of the pancreas with an endocrine component, treated with multiple chemotherapeutic agents, in which the patient survived 37 months after diagnosis.