Objective Mucormycosis is a very rare filamentous fungal infection occurring as a severe complication in neutropenic patients with hematological malignancies. The most frequent sites of infection are lungs and upper airways.

Methods We report on a 16-year-old girl with acute myeloid leukaemia with cerebral infarction due to mucormycotic emboli.

Results The girl received four courses of standard risk chemotherapy (AML-BFM-2004 protocol). After the induction therapy she developed pulmonary infiltrates suspected to be aspergillosis. Liposomal amphotericin B was given, followed by oral voriconazole which was continued during the whole chemotherapy. After the fourth course of chemotherapy febrile neutropenia occurred. Blood cultures revealed klebsiella and antibiotic treatment was initiated. Six days later neutropenia and fever still went on and the girl complained of sudden hemianopsia, headache and nausea. Cranial MRI revealed a cerebral infarction of the right posterior cerebral artery. Angio-MRI ruled out vascular occlusion. No focus of thromboembolisms was found. The clinical course showed continous deterioration with somnolence and cerebral oedema ending in brain death. Autopsy of the brain showed bi-occipital infarction, signs of intracranial pressure and a thromboembolic occlusion of basilar artery, caused by thrombi interspersed with mucorales-type hyphae.

Conclusion Mucorales are known to settle in the internal elastic lamina of arteries and can lead to thromboembolisms. Oral antimycotic agents like triazoles are unable to prevent mucormycosis, amphotericin B is only partially effective. Although mucormycosis is uncommon and diagnosis is difficult it has to be included in the differential diagnosis of fungal infections in neutropenic patients.