Autoimmune hemolytic anemia (AIHA) in pregnancy is uncommon and intractable when it becomes steroid-refractory. Due to lacking well-established guidelines for AIHA in pregnancy, a balance must be weighed between benefits and risks. It is unlikely to undergo clinical trials to investigate the obstetric outcomes of treatments that may have potential toxicities to the fetus. Therefore, observational cases play a critical role in accumulating experience for this minor population. In this report, we describe a pregnant with a history of AIHA for 10 years. The hemolysis was exacerbated during gestation with very severe anemia and increasing splenomegaly, showing no response to glucocorticoid, immunoglobulin, rituximab and high-dose cyclophosphamide. The patient underwent a successful splenectomy and delivered a normal neonate except for low weight. In the follow-up of 2 years, the underlying disease emerged as systemic lupus erythematosus, and the child grew up healthily with a routine immunization schedule.