Polycythemia Vera is characterized by an absolute increase in red cell mass. Patients with PV have a median survival if untreated of approximately 18 months from the time of diagnosis and treated of approximately 18 years. PV-related symptoms include headache, weakness, dizziness, epigastric distress, and pruritus. PV-related signs include hypertension, gout, left upper abdominal quadrant pain, high hematocrit, leukocytosis, and thrombocytosis. Major causes of reduced survival include thrombosis (29%), bleeding (7%), evolution to myelofibrosis (3%), transformation to acute leukemia (23%), and solid tumors (16%). PV patients are stratified for risk of thrombosis by age >60 and history of prior thrombotic events. Therapy for low risk PV includes low dose aspirin and therapeutic phlebotomy to maintain a hematocrit <45% in a man and 42% in a woman. Cardiovascular risk factor modification such as weight loss, control of hypertension and hypercholesterolemia, and smoking cessation are also important adjunctive approaches to all patients with PV. High risk patients are also treated with cytoreductive therapy in the form of hydroxyurea to further reduce the risk of thrombotic complications.