OBJECTIVE To explore the epidemiological profiles of invasive fungal disease (IFD) in hospitalized patients with hematological diseases during 2007-2012. METHODS A total of 419 IFD patients with hematological diseases from January 2007 to December 2012 were reviewed. All of them were analyzed with regards to diagnostic levels, infection sites and various related factors. RESULTS (1) A total of 233 cases (55.61%) were preliminarily identified as IFD, 140 cases (33.41%) had a clinical diagnosis and 46 cases (10.98%) were confirmed cases of IFD. (2) Among 46 confirmed cases of IFD, there were agranulocytosis (n = 43) and aspergillosis infection (n = 36). (3) Respiratory tract was the most frequent infection site in all IFD patients (85.20%) . (4) And chemotherapy-induced agranulocytosis was a major reason for IFD patients with hematological diseases. The number of IFD patients without chemotherapy had a rising trend. (5) The age group of IFD was during 41-60 years old. (6) All of them stayed on antibiotic therapy at the diagnosis of IFD. The numbers of antibiotics were two(205 cases, 48.93%) and three(179 cases, 42.72%). (7) The peak incidence of IFD was recorded in January, July and December. And June was another lower peak. CONCLUSIONS Agranulocytosis is the main reason for IFD patients with hematological disease. The data is important and valuable for the early diagnosis and therapy of IFD patients with hematological disease.