Polycythemia vera (PV) is a myeloproliferative neoplasm characterized by increased red blood cell mass and usually overproduction of granulocytes and platelets and increased spleen size. JAK2V617F mutation is present in more than 95% of PV patients. Bone marrow examination reveals excessive proliferation of erythroid, myeloid, and megakaryocytic elements. The prognosis of PV depends on the severity of the complications occurring during the clinical course. Thrombotic complications are the main cause of morbidity and mortality in PV. Survival is affected whether appropriate therapy is applied during the erythrocytotic phase of the disease. Uncontrolled erythrocytosis poses very high risk for development of thrombosis. Some studies suggest that PV patients have a normal or near-normal life-expectancy. Most studies, however, report excess mortality caused by thrombotic complications and acute leukemia transformation during course of PV. This review aims to highlight the pathogenesis, diagnosis and current management in PV.