We describe a 6-week-old female infant with cutaneous invasive aspergillosis accompanied with hemophagocytic lymphohistocytosis. Aspergillus flavus was isolated from biopsies of necrotic skin lesions on the forehead and scalp; morphologic identification was confirmed by molecular analysis. In vitro antifungal susceptibility testing showed that amphotericin B and triazoles had potent activity. The patient responded well to treatment with intravenous amphotericin B combined with oral posaconazole and local wound care. The hemophagocytic lymphohistocytosis abated after treatment of cutaneous aspergillosis. Both cutaneous invasive aspergillosis and hemophagocytic lymphohistocytosis are severe disorders with high morbidity and mortality requiring prompt diagnosis and treatment.