Objective To analyze and summarize the clinical characteristics,diagnosis and treatment experience of intracranial venous sinus thrombosis(CVST)associated with pegasparaginase(PEG-ASP)in early stage chemotherapy in children with acute lymphoblastic leukemia(ALL).Methods From September 2018 to October 2023,patients with acute lymphoblastic leukemia who were newly diagnosed in Beijing Children's Hospital Affiliated to Capital Medical University and were younger than 16 years old were included in the study.The clinical manifestations,laboratory examination,treatment and prognosis of 4 cases with PEG-ASP CVST were analyzed retrospectively.Results All of them occurred in the early stage of chemotherapy.All were associated with abnormal coagulation function.All were treated with low molecular weight heparin(LMWH).All symptoms disappeared after treatment.Conclusions When unexplained neurological symptoms occur in children with ALL during PEG-Asp chemotherapy,it is necessary to be highly vigilant about the occurrence of CVST,and skull enhanced MRI and MRV should be performed as soon as possible for early diagnosis.LMWH and rivaroxaban were effective,had good prognosis and no sequelae.