This study was aimed to investigate the clinical features and therapy of Ph(+) acute lymphoblastic leukemia (Ph(+)ALL) combined with invasive aspergillosis. A series of examination, including routine blood and bone marrow picture analysis, chest roentgenography, cranial computerized tomography and detection of cell genetics etc were carried out for a Ph(+)ALL patient combined with invasive aspergillosis. This patient received chemotherapy with DVCP, idarubicin and imatinib mesylate and was treated with sporanox and amphotericin B (Amb; including Amb-L) and cerebrotomy for drainage because the invasive aspergillosis occurred during myelosuppression. The results showed that patient gained complete remission and the invasive aspergillosis was controlled successfully. It is concluded that patient with Ph(+)ALL has poor prognosis despite intensive conventional chemotherapy, imatinib mesylate may prove to be an effective treatment for Ph(+)ALL. Because detection rate of the fungus is very low, itraconazole in combination with surgical excision of focus is the best treatment of lung and brain invasive aspergillosis.