Ph-positive acute myeloblastic leukemia (AML) developing in a treated case of Wegener''s granulomatosis is reported. The patient was a 70-year-old white male who received cyclophosphamide (150 mg/day) starting in July 1978; in May 1984 the patient was diagnosed as having AML, following a diagnosis of myelodysplastic syndrome lasting 3 months. Cytogenetic study of bone marrow cells at the diagnosis of the myelodysplastic syndrome revealed a standard ph translocation in addition to numerical chromosome changes [i.e., 45,XY-5,-7,-8,+19,+mar/45,XY,-5,-7,-8,+19,+mar,t(9;22)(q34;q11)]. The events in this case suggest a strong possibility of the Ph-positive AML being a secondary disease related to prior cyclophosphamide therapy.