Objective To investigate the risk factors of death in extremely preterm infants (EPI). Methods The clinical data of EPI hospitalized in the neonatology department from January 1, 2018 to December 31, 2021 were retrospectively analyzed. According to the clinical outcome at the time of discharge, the patients were divided into the survival group and the death group, and the clinical characteristics between the two groups were compared to analyze the risk factors of EPI death. Results A total of 114 EPI patients (58 boys and 56 girls) were included. The median gestational age was 27.0 (26.0-27.6) weeks, the mean birth weight was (921±179) g, and 24 (21.1%) died. There were no significant differences in the proportions of advanced maternal age, cervical insufficiency, gestational diabetes mellitus, premature rupture of membranes, prenatal use of glucocorticoids and antibiotics between the two groups (P>0.05). Multivariate logistic regression analysis showed that birth weight ≤750g, severe neonatal respiratory distress syndrome, pulmonary hemorrhage within 1 week after birth, insulin use within 1 week after birth, and septic shock were independent risk factors for EPI death (P<0.05). Conclusions Body weight ≤750g, severe neonatal respiratory distress syndrome, septic shock, and pulmonary hemorrhage within 1 week after birth, or insulin use within 1 week after birth have a higher death risk in EPI.