BACKGROUND: In patients with hematologic diseases, surgical resection can be recommended for definite diagnosis, curative treatment, and prevention of complications or redevelopment of invasive pulmonary fungal diseases (IPFD). The purpose of this study was to investigate the outcome of surgical resection for IPFD in patients planned to undergo subsequent chemotherapy (CTx) or hematopoietic stem cell transplantation (HSCT) for acute leukemia. MATERIALS AND METHODS: We reviewed the medical records of adult patients with acute leukemia who underwent surgical resection for IPFD which developed during the neutropenic period after CTx. RESULTS: From January 2004 through August 2008, a total of 15 patients (8 males and 7 females with median age of 49 years) underwent surgical resection. All patients were treated by elective surgical resection of residual IPFD lesion before subsequent CTx or HSCT. The median diameter of the main lesion was 66 mm (range, 33-98 mm). Pericardial adhesion due to local invasion of pulmonary lesion was observed in one patient. Lobectomy was performed in 13 cases, lobectomy with wedge resection in 1 case, and segmentectomy with wedge resection in 1 case. Air leakage was complicated in 2 patients. Thirty-day mortality after surgical resection was 0%. After subsequent CTx or HSCT, IPFD redeveloped in 5 patients. However, the overall mortality was not different between the groups with or without the redevelopment of IPFD. Also, mortality attributable to IPFD was only 6% (1/15) during the overall follow-up period (median 184 days, range 58-1,251 days). CONCLUSIONS: In patients planned to receive subsequent CTx or HSCT for acute leukemia, surgical resection combined with medical therapy for IPFD could be considered for those who have significant residual lesion. Further study will be needed to determine whether surgical resection can shorten the duration of medical treatment and improve survival outcome.