A fatal case of craniofacial zygomycosis caused by Apophysomyces elegans in a 52-year-old man was diagnosed by the presence of broad aseptate, branched hyaline hyphae in tissue from paranasal sinuses and surrounding areas, and isolation of the fungus from the same tissue. The patient suffered from idiopathic myelofibrosis as underlying disease, he was thrombocytopenic and was mildly hyperglycaemic. The infection represents the second case of craniofacial zygomycosis due to A. elegans.