Pancreatic exocrine cancer (PEC) is a challenging disease with a very low of curability rate, even when disease is at early stage and surgically treated, with dismal 5-years survival rates. Advanced disease is always a fatal disease and case of long lasting survivors are very few and doubts on the faithfulness of the diagnosis are more than justifiable. There are some benign illness that can mimic radio-logically and clinically an advance pancreatic cancer? We describe a case of clinically and radio-logically advanced pancreatic cancer who had non diagnostic fine needle aspiration biopsy (FNAB) with a long lasting remission after a short course of therapy with gemcitabine. The atypical outcome after systemic therapy and doubts on reliability of the clinical diagnosis carry us to review literature in search of similar cases, under the point of view of positive outcome and misleading clinical presentation.