Objective To investigate the clinical and laboratory characteristics of acute myeloid leukemia (AML) with t (16;21)(p11;q22) translocation.Methods Two patients diagnosed by morphology,cytochemical stain,immunology,cytogenetics and genetic testing.Similarities and differences of clinical characteristics and laboratory examination were analysed,along with a review of the literatures.Results According to the FAB classification,one patient was M4 and the other one was M1.The cytogenetic aberrations were 46,XY,t(16;21)(p11;q22)[16]/47,XY,t(16;21)(p11;q22),+21[4] of ease 1 and 46,XX,t(16;21)(p11;q22)[20] of case 2.Cytophagocytosis and CD56 antigen expression were found in both cases.The prognosis was poor in both cases.Conclusions AML with t(16;21)(p11;q22) is a specific type,which has unusual characteristics of morphology,immunology,cytogenetics,clinical feature.The prognosis of the patients is poor,so stem-cell transplantation maybe the only and the first choice of treatment.