Objective To explore the clinical characteristics and prognostic factors of pediatric acute myeloid leukemia (AML) with monosomy 7 (-7) and deletion of the long arm of chromosome 7 (7q-). Methods A retrospective study was conducted on the clinical data, treatment, and prognosis of children with -7/7q- AML who were admitted to the Department of Pediatrics at Peking University People's Hospital from January 2010 to December 2024. Results A total of 869 children with AML who had complete karyotype data were included, of whom 32 (3.7%) had -7/7q- chromosomal abnormalities. There were 20 males and 12 females, and the median age at diagnosis was 6 years. Six children (19%) had isolated -7; 2 (6%) had isolated 7q-; and 24 (75%) had additional chromosomal abnormalities. After induction chemotherapy, complete remission (CR) was achieved in 16 children (50%). At the last follow-up, 15 children (47%) had died and 17 (53%) were alive. The 3-year disease-free survival (DFS) rate was (54.1±0.1)%, and the 3-year overall survival (OS) rate was (52.6±0.1)%. The multivariable analysis showed that hematopoietic stem cell transplantation (HSCT) was an independent prognostic factor for DFS (HR=0.17, 95%CI: 0.04-0.62, P=0.008) and OS (HR=0.16, 95%CI: 0.04-0.59, P=0.006), with better outcomes in children who underwent HSCT. Conclusions The incidence of -7/7q- chromosomal abnormalities in children with AML is 3.7%. Additional chromosomal aberrations are common, and the CR rate after induction chemotherapy is low. HSCT is associated with improved prognosis and survival.