Monosomy 7 (؊7) and deletion 7q [del(7q)]are rare in childhood acute myeloid leukemia (AML). We retrospectively collected data on 258 children with AML or refractory anemia with excess blasts in transformation (RAEB-T) and ؊7 or del(7q) with or without other cytogenetic aberrations [؎ other]. Karyotypes included ؊7 (n ؍ 90), ؊7 other (n ؍ 82), del(7q) (n ؍ 21), and del(7q) other (n ؍ 65). Complete remission (CR) was achieved in fewer patients with ؊7 ؎ other compared with del(7q) ؎ other (61% versus 89%, P < .001). Overall, the 5-year survival rate was 39% (SE, 3%). Survival was superior in del(7q) ؎ other compared with ؊7 ؎ other (51% versus 30%, P < .01). Cytogenetic aberrations considered favorable in AML [t(8;21)(q22;q22), inv(16)(p13q22), t(15;17)(q22;q21), t(9;11)(p22;q23)] (n ؍ 24) were strongly associated with del(7q) and a higher 5-year survival rate compared with del(7q) without favorable cytogenetics (75% versus 46%, P ؍ .03). Patients with ؊7 and inv(3),؊5/del(5q), or ؉21 had a 5-year survival rate of 5%. Stem cell transplantation analyzed as a time-dependent variable had no impact on overall survival. However, patients not achieving CR had a 31% survival rate after stem cell transplantation. Childhood AML with chromosome 7 aberrations represents a heterogeneous group of disorders with additional cytogenetic aberrations having a major prognostic impact which should be reflected in future riskgroup stratification. (Blood. 2007;109: [4641][4642][4643][4644][4645][4646][4647]