Neonatal hypertension is a rare and complex condition, with an incidence rate of 0.2% to 3%. It can be resulted by several risk factors such as renal diseases, use of several drugs, neurologic, cardiac, respiratory and endocrine causes. Gestational age, birth weight and conceptual age are the most important risk factors. Renal diseases can be found in about 25% to 50% of hypertension at neonatal intensive care units (NICU). The diagnostic of neonatal hypertension is not easy because its symptoms are often non-specific. Hypertension usually discovered on routine monitoring of vital signs. Physical examination and clinical history of neonates along with laboratory diagnostic tests and imaging studies are necessary for the identification of the disease. Calcium channel blockers, angiotensin-converting enzyme (ACE) inhibitors, α- and β-blockers, vasodilators, and diuretics are the most commonly used antihypertensive agents in NICU. Surgical interventions are uncommon; however, they are valuable in cases with secondary hypertension. This review aimed to discuss recent data on the etiology, accurate diagnosis and treatment of neonatal hypertension.