Objective　To investigate the clinical features of abdominal tumor with paraneoplastic neurological syndrome (PNS) in children. Methods　Clinical features, CSF test, peripheral blood test, EEG, brain MRI and abdominal imaging test of 3 cases with PNS were retrospectively reviewed. Results　The three patients (two boys and one girl) were diagnosed as PNS with onset of neurological symptoms. The average age of PNS patients were 3.7±1.7 years old. The level of LDH in peripheral blood was elevated. More slow waves occurred in EEG. Abdominal imaging test showed abdominal tumors. The white cell count of CSF test was higher than normal and oligoclonal protein was found positive in one case. Brain MRI showed corresponding changes in two cases. Conclusions　The onset of PNS was dormant. Early diagnosis through CSF tests, LDH, brain MRI and abdominal imaging tests can help abdominal tumor patients to get early treatment.