Background/aim To evaluate the incidence, clinical features, risk factors, and prognosis of central nervous system (CNS) involvement in patients with acute myeloid leukemia (AML). Materials and methods All AML patients who were admitted to Hacettepe University hospital between 2000 and 2021 were evaluated. The medical records of 548 AML cases were retrospectively analyzed. Results The frequency of CNS involvement was 2.4% (n = 13) at diagnosis and 4.6% (n = 25) at diagnosis or during follow-up. Parenchymal involvement was seen in 5 patients, leptomeningeal involvement was seen in 11 patients. Three patients had both leptomeningeal and parenchymal involvements, and 6 patients had optic nerve or ocular involvement. In univariate analysis, younger age and extramedullary involvement at diagnosis were associated with CNS disease at diagnosis, and extramedullary involvement at diagnosis was associated with CNS disease during follow-up. In multivariate analysis; younger age and extramedullary involvement at diagnosis were associated with CNS disease at diagnosis and during follow-up respectively. Median overall survival was 5.4 months in patients with CNS disease at diagnosis and 16.9 months in patients with CNS disease during follow-up and 16.2 months in patients with no CNS disease. Conclusion CNS disease is a rare complication of AML. Younger age and extramedullary involvement at diagnosis are risk factors for CNS involvement.