Zygomycosis is an invasive fungal infection with extremely high mortality caused by filamentous fungi which belong to Class Zygomycetes (Rhizopus spp., Mucor spp., Cunninghamella spp., etc). Despite of the similarities of the ecological characteristics and of the patients' backgrounds, zygomycosis is much rarer than invasive aspergillosis. In addition to well known immunosuppressive risk factors (hematological malignancy, hematopoietic stem cell or solid organ transplant, prolonged neutropenia, corticosteroid, etc), diabetic ketoacidosis, iron overload, and administration of deferoxamine are specific factors predisposing zygomycosis. Rhinocerebral, pulmonary and disseminated disease is characteristic forms. The mainstay of the treatment is surgical resection, reversal of immunosuppressive factors, and administration of high-dose amphotericin B or its liposomal formulation. Because of the difficulty of culture detection and the absence of reliable serological diagnostic methods, premortem diagnosis and no delaying of effective treatment remain a challenge to physicians.