The objective of this study is to present an unusual case of Fanconi anemia (FA) with an initial presentation of abdominal rhabdomyosarcoma followed by acute myeloid leukemia (AML). Although epithelial cancers are more often seen in FA patients, rarities such as osteosarcomas, soft-tissue tumors and brain cancers have been reported in the literature. The present case was treated with VACA treatment (vincristine, actinomycin D, cyclophosphamide and adriamycin) and radiotherapy following initial assessment through surgery and histopathological evaluation. A second malignancy AML developed 17 months after completion of initial treatment and the patient died at 2 months remission and 6 months survival following the diagnosis of AML. [Turk J Cancer 2007;37(4):154-157]