Mixed acinar-endocrine carcinoma of pancreas is a very rare tumor. We report a 60s female patient with pancreatic mixed acinar-endocrine carcinoma and liver metastasis. The patient admitted for further examination of pancreatic head mass. Computed tomography scan of abdomen showed a large tumor in pancreatic head and liver tumor. Angiography revealed a presence of embolism in portal vein. We conducted a pylorus-preserved pancreatoduodenectomy with resection of the portal vein on the diagnosis of acinar cell carcinoma by fine needle aspiration biopsy. Pathological examination showed a mixed acinar-endocrine carcinoma. Following the operation, the liver metastasis was controlled with various loco-regional cancer therapies.