Aspergillosis of the central nervous system presents a challenge faced with the problems arising from the establishing the diagnosis, the low efficiency of treatment, and high mortality rates (about 95% as shown by some data). This paper presents a clinical case of a patient with aspergillosis-induced central nervous system lesion verified by autopsy. Possible errors in the diagnosis and treatment of the patient are analyzed. The literature data including clinical cases, etiopathogenesis, and clinical manifestations and its possible complications are presented. Groups of patients at risk for invasive aspergillosis are considered. The paper gives the data available in the foreign literature on the methods and efficiency of treatment and mortality rates in different groups of patients. The urgency of the problem in the diagnosis and treatment of cerebral aspergillosis rises with the increased number of patients with immunological disorders due to infectious diseases (HIV), social (drug addiction, alcoholism), environmental, and other factors.