RESUMEN
Infantile liver failure syndrome-2(ILFS2)is a rare autosomal recessive disorder caused by neuroblastoma amplified sequence(NBAS)gene mutation, manifested as recurrent acute liver failure(ALF)with fever/infection-related pathogenesis.First-onset ALF is common in infants or early childhood(8 months to 3 years of age). The main characteristic of this disease is that the liver function can be recovered completely in the interval, and the definitive diagnosis is based on the identification of NBAS gene mutation in gene analysis.Until now, the pathogenesis of ILFS2 is not yet fully understood.Patients can be treated by supportive treatment clinically, while liver transplantation is the only treatment option currently available for patients with end-stage ALF.This review will focus on the recent progress in the pathogenesis and treatment of ILFS2.