Zygomycosis constitutes the third leading cause of invasive fungal infections following aspergillosis and candidosis. Patients with haematologic malignancies or haematopoietic stem cell transplantation are particularly susceptible to zygomycosis. Neutropenia represents the most important pathogenic mechanism influencing the onset and outcome of zygomycosis. Neutrophils cause a lesion of the fungal wall with subsequent destruction by macrophages. They also enhance the activity of antifungal drugs against Zygomycetes. Strategies that aim to increase neutrophil count and function, such as granulocyte colony stimulating factor and granulocyte transfusion, could play an important role in the management of this life-threatening infectious complication.