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Background  The diagnosis of pancreatic cystic neoplasms has become more accurate recently. In some cases, however, doubt remains regarding the lesion’s malignant potential. CA 19–9 has long been identified as a reliable biomarker in differentiating pancreatic benign and malignant lesions, especially in non-jaundiced patients.    Case report and discussion  We report a case of a young female who presented with a mucinous lesion in the tail of the pancreas and a serum CA 19–9 over 1,000,000 U/mL. She was taken to surgery and had a distal pancreatectomy and splenectomy. Pathology reports showed only a mucinous cystadenoma. After 1 year of follow-up, her serum CA 19–9 was normal. Following that, the work-up in these lesions, the role of the biomarker in pancreatic adenocarcinoma and in the differentiation between benign and malignant lesions is discussed.

Pancreatic mucinous cystadenoma with serum CA 19–9 over 1,000,000 U/mL: a case report and review of the literature