A 67-year-old male patient with a pancreatic carcinoma and hepatic metastases was admitted with progressive dyspnea and anuria. Previously he had received five cycles of a palliative chemotherapy with gemcitabine and responded well with a reduction of the tumor mass. The laboratory results showed a distinct anemia, thrombocytopenia, an increase in creatinine and lactate dehydrogenase levels. With an additional finding of 21 per mille fragmentocytes in the periphery blood smear, we diagnosed a thrombotic microangiopathy (TMA). Until now the literature lacks well-defined therapy standards for this known but rare condition of gemcitabine. In a few case reports addressing the related microangiopathy of thrombotic thrombocytopenic purpura (TTP), which is characterised by a significant reduction of the von Willebrand factor (vWF) cleaving serum protease ADAMTS-13, encouraging results have been achieved by an immediate plasma exchange. Though ADAMTS-13 activity was not relevantly reduced in our patient, we still observed a rapid improvement of the clinical features as well as of LDH, thrombocytes and fragmentocytes under plasma exchange. The patient was discharged after one month in good clinical condition. Interestingly, during follow-up for further 21 months we found a continued stable status of the pancreatic carcinoma without any cytostatic therapy. In summary, this case provides evidence that the use of plasma exchange therapy in gemcitabine-associated TMA is justified.