Refractory nephrotic syndrome accounts for about 30%-50% of primary nephrotic syndrome, including steroid-dependent nephrotic syndrome, steroid-resistant nephrotic syndrome, and frequent relapse nephrotic syndrome, with characteristics of long durations and easy relapses. If the state of disease cannot be controlled with proper treatment, it might progress to chronic renal failure even with serious complications which greatly affects the patients′ quality of life and might be life-threatening. Although immunosuppressive therapies such as corticosteroids plus alkylating agents have been adopted as classic regimens, the potential gonadal toxicity, bone marrow suppression, and carcinogenic adverse effects limit their clinical applications. In recent years, tacrolimus, a novel immunosuppressive agent, has shown relatively good effects in the treatment of refractory nephrotic syndrome at home and abroad. This article presented the new progress in the efficacy and safety of tacrolimus for the treatment of refractory nephrotic syndrome.