Abstract: 　Objective　To investigate the clinical features, hepatic pathology, and gene mutations of the drug-induced liver disease (DILD) in children. Methods　The clinical manifestations and laboratory results from four children with DILD were retrospectively analyzed. The relevant literatures were reviewed. Results　Four children with DILD all had associated medication history before onset. The courses were 5-90 days. The first symptom was various in common with jaundice, yellow  urine, liver function abnormality, and hepatomegaly. Liver biopsy was performed in 2 patients. The hepatic pathological manifestation mainly showed extensive water degeneration, focal necrosis, and inflammatory cells in portal area. Ultrastructural pathology showed numerous glycogen storage, bile pigment particle deposition, and lymphocyte infiltration. Gene mutation was detected in 2 patients and their parents. The DNA sequencing of the ABCB11 gene showed heterozygous mutations in the exon of No.13, 13311 T>C(V444A) and No.21, 2594 C>T(A865V) in one patient. Gene mutation was not detected in another patient. The literature review showed that clinical classification was mainly composed of hepatocellular type and antibiotics were the most common medicine to induce DILD in domestic children. Meanwhile, antipyretic-analgesic was the most common medicine to induce DILD in foreign children. ABCB11 gene mutation had been found more than 100 kinds. The V444A mutation was the most common site. Conclusions　The first symptoms of children with DILD are various. Medication history before onset, hepatic pathology, and detection ABCB11 gene were helpful to diagnose DILD.