Abstract: The morbidity and mortality of patients with invasive fungal disease (IFD) and hematological malignancies are high. Early diagnosis and treatment can effectively reduce the incidence of IFD. For a definitive diagnosis of IFD, various diagnostic tools must be used, including routine fungal culture, serological and molecular biological examinations, and histopathological and radiological examinations. As no single indirect test, including radiological methods, can provide a definitive diagnosis of an invasive fungal infection, a combination of different diagnostic methods and clinical manifestations of the patients can be used to determine the final diagnosis of IFD. However, even if clinical diagnosis does not provide definitive evidence of IFD, early antifungal therapy is mandatory for eligible patients. When determining the clinical treatment plan, the clinical characteristics, laboratory and radiological examination results, pharmaco-pharmacological characteristics, and economic burden of the patients should be considered. This review intends to summarize the latest recommendations for the diagnosis and treatment of invasive fungal infections in patients with oncohematological diseases.