Objective To explore the clinical features and pathology of drug-induced vanishing bile duct syndrome (VBDS) in children. Method The clinical data of drug-induced VBDS in 3 children from January 2019 to December 2019 were collected and analyzed in combination with the literature. Results Three children ( 1 girl and 2 boys) aged from 9 months to 8 years had jaundice cholestasis with pruritus as main clinical manifestations. All the patients were treated with liver protection, anti-jaundice and immunosuppression. After 3 months of treatment, the clinical symptoms and biochemical indexes of 2 patients were basically recovered. One patient still presented mild jaundice cholestasis after 1 year of treatment and was still in follow-up. Conclusion? Early identification of drug-related VBDS, discontinuation of related drugs and active treatment can improve the prognosis.