A 34-year-old man was referred to our hospital for small lung nodules. An imaging study including a computed tomographic scan and magnetic resonance imaging showed bilateral lung tumors and huge mass arising from the lower pole of the right kidney as well. The patient underwent right radical nephrectomy in May 2002. Pathological examination revealed that the tumor was pheochromocytoma, which developed from extra-adrenal tissue. In 6 cycles of CVD systemic chemotherapy (Cyclophosphamide 750 mg/m2, Vincristine 1.4 mg/m2, Dacarbazine 600 mg/m2), the lung tumors were decreasing in size, and were removed by a surgical procedure in January 2003. Pathological examination revealed that the lung tumors were pheochromocytomas metastasized from the primary tumor. He is alive without evidence of disease 5 months after the lung operation.