Pediatric leukemia is a common cause of disease-related fatality in children aged 1 to 10 years. This disease is usually treated with a combination of chemotherapy, radiation therapy, and bone marrow transplantation. With advancements in cancer treatment, approximately 70-80% of pediatric cancer patients have been cured with these aggressive combination therapies. However, oral and dental infection, mucositis, and necrosis of tissues can occur during the cancer treatment, which may lead to morbidity and an inferior quality of life for pediatric patients with leukemia. Early detection and radical treatment reduce the risk of oral infection and its associated systemic complications. Moreover, dental treatment such as extraction or preventive treatment of teeth should be considered before the start of the cancer treatment. However, it is difficult to provide dental treatment to pediatric patients with leukemia in usual settings due to their uncooperative behavior and/or the presence of psychiatric disorders. Therefore, general anesthesia during dental treatment is often recommended for these patients. In this paper, we reviewed the anesthetic management and perioperative complications in pediatric patients with leukemia receiving general anesthesia.