Hemophagocytic lymphohistiocytosis (HLH) is characterized by fever, 
splenomegaly, jaundice, and pathologic findings of hemophagocytosis in bone 
marrow or other tissues such as the lymph nodes and liver. Pleocytosis, or the 
presence of elevated protein levels in cerebrospinal fluid, could be helpful in 
diagnosing HLH. However, the pathologic diagnosis of the brain is not included 
in the diagnostic criteria for this condition. In the present report, we 
describe the case of a patient diagnosed with HLH, in whom the brain pathology, 
but not the bone marrow pathology, showed hemophagocytosis. As the diagnosis of 
HLH is difficult in many cases, a high level of suspicion is required. Moreover, 
the pathologic diagnosis of organs other than the bone marrow, liver, and lymph 
nodes may be a useful alternative.