Polycythemia vera is a clonal disorder involving a multipotent haematopoietic progenitor cell in which phenotypically normal red cells, granulocytes and platelets accumulate in the absence of a recognizable physiologic stimulus. It is one of the common form of chronic myeloproliferative diseases. A few patients present with its actual clinical manifestations. We present a case where polycythemia vera presenting as a paradox in the form of anemia.

On admission, the patient presented with giddiness which was of acute onset and black colored stools since one week. OGD was normal. Esophageal ultrasonography showed duodenal and peripancreatic collaterals. CT Abdomen showed chronic portal vein and superior mesentric vein thrombosis with mesentric, peripancreatic ,paraduodenal collaterals. Excluding causes of thrombosis, erythropoietin levels was low, and polycythemia was then suspected. JAK2 mutation was positive, and the patient was diagnosed with polycythemia vera.