Hemophagocytic syndrome is a rare life-threatening clinical disease, characterized by pancytopenia and liver dysfunction, and seriously damages the function of blood coagulation of the patient. We here report a case of 6 years old patient with hemophagocytic syndrome suffered from significant aspiration during induction of anesthesia and the gastric contents which lead to aspiration was thought to be the unknown dated spontaneous bleeding in the stomach. We detailed describe the clinical treatment of the patient and the disease outcome, the anesthetic key points of the patient with hemophagocytic syndrome, and we systemic reviewed the regurgitation and aspiration relevant to anesthesia, including the evaluation of risk and corresponding treatment, the choice of anesthesia way and clinical strategy after aspiration.