A previously healthy 19-year-old man developed rapidly progressive invasive rhinocerebral zygomycosis due to Apophysomyces elegans. He required extensive surgery and prolonged treatment with high-dose i.v. amphotericin B for cure. This is only the third reported case of acute invasive rhinocerebral zygomycosis in an otherwise healthy patient and the first reported case of infection due to A. elegans in any patient. We review the literature and clinical spectrum of rhinocerebral zygomycosis in otherwise healthy patients and discuss the recently recognized association between A. elegans and zygomycosis in immunocompetent patients.