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Acute cardiorenal syndrome (CRS) is often observed in patients with acute kidney injury (AKI) in the cardiac intensive care unit and is reported to be associated with poor prognosis. Volume disorder or re-distribution, renin-angiotensin-aldosterone system activation, and neurohormonal and sympathetic nervous system activation have been suggested to be related to the occurrence of acute CRS. There is a lack of biomarkers that can identify changes in renal function in patients with acute CRS. Evidence-based medications are limited in the management of acute CRS in AKI. Therefore, we reviewed the epidemiology, pathophysiology, clinical assessment, and treatment of acute CRS in AKI.