Since the beginning of 2022，acute severe hepatitis of unknown aetiology in children has attracted great attention. As of June 22，a total of 920 probable cases have been reported from 33 countries with a high proportion of severe cases. To prepare for the management of possible cases，the National Health Commission of the People's Republic of China issued the Guidelines for acute severe hepatitis with unknown aetiology in children （Trial）. Based on the published data，this paper summarized its definition，possible etiology and clinical features from a perspective of pediatrician，and discussed the strategies of evaluation and treatment，in order to enhance the cognition of pediatric clinicians for this disease.