We report a case of acute myeloid leukemia (AML) with trisomy 10 as the sole abnormality. We have observed this case among 202 de novo and untreated AML examined cytogenetically in our laboratory during the last 10 years. The patient was an adult man diagnosed with AML-M2. An interesting morphologic finding was the presence in light microscopy and ultrastructural studies of prominent giant "Chediak-Higashi-like" granules in some of the leukemic cells. Cell marker studies showed positivity for CD7 and CD33, as seen in the six previously reported cases. The prognosis has been moderate-good, with a survival of 33 months. Trisomy 10 in AML appears to be a rare recurring numeric chromosomal abnormality and probably linked to a myeloblast subset with a CD33+ and CD7+ phenotype.