Polycythemia vera (PV) is a condition that originates from stem cells and this disorder is affiliated with chronic myeloproliferative neoplasms (MPNs). It is highlighted by malignant neoplastic proliferation of the trilineage elements of bone marrow. Even though it is insufficient of establishing a definitive diagnosis, it is distinguished from other myeloproliferative neoplasms by increased red blood cell mass. Since the course of this disease is insidious and usually picked up incidentally, prompt diagnosis and proper management reduce the risks of fatal sequelae. The goal of this study is to review the literature and enhance the understanding and awareness of polycythemia. We reviewed the literature for polycythemia; clinical manifestations and approach to diagnosis. Articles were chosen from the PubMed database, and selected studies were subjected to a thorough review. Because of its gradual onset, polycythemia Vera is difficult to diagnose, however, it should be suspected in people having an increased red blood cell mass. Abnormally high hemoglobin or hematocrit, combined with a clinical presentation like splenomegaly, thrombocytosis, and leukocytosis, highly suggests it. As quickly as possible, a referral to a hematologist for bone marrow aspiration and therapy should be arranged. Consequently, with correct therapy and monitoring, the risk of thromboembolism can be reduced.