Objective To investigate the biological characteristics of biphenotypic acute leukemia (BAL) with t(8;21)(q22;q22) in children. Methods The characteristics of cellmorphology, immuno-phenotypes, chromosome karyotype and molecular biology of 7 cases of childhood BAL with t (8; 21) (q22;q22) were analyzed. The control group included 30 cases of acute myeloid leukemia(AML) without t(8 ;21) translocation detected during same period. Results The percentage of BAL with t (8 ;21) (q22;q22) in the 190 cases of the AML was 3.7% (7/190) . The BAL with t(8;21) of which the expression was t (8;21) (q22;q22) that the bone marrow cell morphology showed the AML -M2. The initial cells all increasd evidently in the classification all P < 0.01). The immunophenotypes were all in the category of the expressions of the myeloid markers with B-lymphoid. CD34 had high expression of positivi-ty. There were changes of the chromosomal with t ( 8 ;21) ( q22; q22) also with the complexly depletion or translocation. The detection results of the confluent genes AML1/ET0 were positive. The total complete remission (CR) rate was 71.4% . It achieved good response to combined chemotherapy targeting to both myeloid and lymphocytic leukemia. Conclusion BAL is a rare type of acute leukemia. The unfavorable indicators of BAL may be concerned abnormal karyotypes, high expression of positivity of CD34. Combined chemotherapy targeting to both myeloid and lymphocytic leukemia contribute to improving survival.