Cerebral aspergillosis is a devastating disease in patients with a compromised immune system. A unique case of a male infant with corticosteroid-resistant nephrotic syndrome complicated by pulmonary and cerebral aspergillosis is described. The patient rapidly developed coma and neurological symptoms and died soon thereafter. Central nervous system aspergillosis was diagnosed radiologically and by detection of Aspergillus DNA and antigen (galactomannan) in blood and cerebrospinal fluid. Moderate immunosuppressive therapy and antifungal phagocytic dysfunction due to nephrotic syndrome per se may have contributed to the occurrence of invasive aspergillosis in our patient. Awareness of this serious complication and early application of diagnostic procedures and antifungal therapy may improve the dismal outcome.