Objective To investigate clinical characteristics, early diagnosis and therapy of children with fulminant Wilson disease (FWD). Method The clinical data from two children were retrospectively analyzed. Related articles were reviewed. Results Two female patients with FWD aged 5 and 8 years old, presented with acute jaundice, hemoglobinuria, liver failure, hepatic encephalopathy, renal failure, myocardial injury, organ bleeding, hepatomegaly and K-F ring positive, which were in line with rapid diagnostic criteria. Exons of ATP7B gene were sequenced and a p.P992L, a p.A1063V and a c.2764del9 were found in the two patients. Conclusions Any child present with acute liver injury should be considered the possibility of FWD, which can refer to Wilson index, rapid diagnostic criteria and other data to help diagnose.