The influence of ABO incompatibility on transplant results in juvenile aplastic anemia (SAA) children receiving hematopoietic stem cell transplantation (HSCT) is debated, and no published data are available. This study involved 85 children with AA who received hematopoietic stem cell transplants from donors who were ABO-compatible (n = 38), major ABO-incompatible (n = 17), minor ABO-incompatible (n = 21), and bi-directionally incompatible (n = 9). Except for a statistical difference in acute graft-versus-host disease (aGVHD), the four groups did not have significantly different chronic GVHD, overall survival, and neutrophil and platelet implantation rates, and no pure red cell aplasia or passenger lymphocyte syndrome. Therefore, we inferred that ABO blood group-incompatible donors do not significantly affect HSCT outcomes and are not a barrier.