Brain death is defined as the complete and irreversible loss of cerebral and brainstem function while the heart continues to beat. Unlike cardiac death, brain death is associated with medical, legal, and ethical issues. Common causes of neonatal brain death are perinatal asphyxia, birth trauma, cerebral infection, malformations, severe intracranial hemorrhage, and metabolic diseases. Although a diagnosis of brain death is usually based on clinical criteria, it is not well defined in neonates, especially in preterm babies. Furthermore, there are no guidelines on life expectancy, prolonging life care, or discontinuation of life support in brain dead newborns in Korea. We report a case of brain death due to hypoxic ischemic encephalopathy in a near-term newborn, present the need to establish guidelines for brain death in newborns based on national standards, and discuss further directions about end of life treatment after brain death.