We describe a case of acute leukemia in a child with an unusual immunophenotype and a novel cytogenetic abnormality. The leukemia blasts expressed myeloid, natural killer and B-lineage associated antigens. Cytogenetics showed the presence of a novel unbalanced chromosomal translocation, der(19)t(12;19)(q12;p13.3). The patient achieved and maintained remission with myeloid-directed chemotherapy. The differential diagnosis of the immunophenotype and the potential fusion genes are discussed.