Pancreatic acinar cell carcinoma (PACC) is a malignant epithelial tumor arising from pancreatic epithelium, which is rare in clinical practice due to low incidence, and it has no specific clinical manifestations. Previous studies have reported that PACC accounts for merely 1%-2% of exocrine pancreatic tumors and 0.44% of pancreatic malignant tumors. This disease is challenging to diagnose, has low resection rates, and easily metastasizes. However, the prognosis is better than that of pancreatic ductal adenocarcinoma.Understanding and mastering clinical diagnosis and treatment of PACC can improve diagnostic rate and clinical prognosis.