Background: Polycythemia vera is considered a malignant stem cell disorder and its patients usually experience troublesome symptoms and increased risk of mortality and morbidity. Appropriate diagnosis and management is cardinal in such cases to avoid disease complications and improve the patient’s quality of life. Objective: To review the literature that discussed the evaluation and management of cases of polycythemia vera. Method: PubMed database was used for articles selection, and the following keys were used in the mesh ((“polycythemia vera"[Mesh]) AND (“Management” [Mesh]) OR (“Evaluation"[Mesh])). Conclusion: Life-expectancy in polycythemia vera is significantly lower than that of the age- and sex-matched general population. Moreover, despite polycythemia vera is considered as a malignant hematopoietic disorder, many cases do not require chemotherapy because they usually have stable and subtle clinical course. Recent recommendations suggest that all polycythemia vera patients require phlebotomy and once- or twice-daily aspirin if there are no contraindications.