Acute hemolysis induced by linked blood line during hemodialysis is one of the life-threatening complications. The occurrence rate is rare. However, if the kinked blood line cannot be detected and resolved promptly, the outcome is severe due to the complications including hyperkalemia, acute pancreatitis and even death following acute hemolysis. This paper focuses on the mechanism, diagnosis, prevention and management of acute hemolysis based on review of the literature about hemolysis events, in order to strengthen the recognition and the aware of hemolysis risk induced by kinked blood line in dialysis staffs, to protect the patients from hemolysis events, and to ensure safety and optimal efficiency for hemodialysis patients.