Abstract: Objective To study the clinical manifestation, treatment, prognosis, and delivery outcome of a case with hemophagocytic syndrome in pregnancy. Methods Clinical data, delivery outcome and follow-up data of a pregnant woman complicated with hemophilia syndrome treated in the obstetrics department of the first medical center of Chinese PLA General Hospital in May 2018 were analyzed. Results A 29 years old female presented with fever at 31 weeks 2 days gestation for 13 days. The patient underwent emergency cesarean section under general anesthesia on May 8, 2018, and then was transferred to ICU. She was diagnosed as pregnancy with hemophagocytic syndrome, and received symptomatic treatment, dexamethasone and etoposide after operation. During the follow-up, the patient recovered gradually. Conclusion Pregnancy complicated with hemophagocytic syndrome needs to be diagnosed as soon as possible, and standard treatment should be given after termination of pregnancy to achieve good prognosis.