Kikuchi-Fujimoto disease was initially described as a self-limiting histiocytic necrotizing lymphadenitis in Japan in 1972, and is predominantly observed in women under the age of 30 year and in Asian populations. The pathogenesis is still poorly understood but is thought to include infections, and autoimmune and neoplastic diseases. The most common clinical manifestations are fever and painless cervical lymphadenitis. Diagnosis is based on the histopathological findings, characterized by focal necrosis in the paracortical region with abundant karyorrhexis, aggregates of atypical mononuclear cells around the zone of necrosis, absence of neutrophils and plasma cells, and usually intact lymph node capsule. There is no specific therapy for the condition, and aseptic meningitis can occur as one of the complications. Here, we report the case of a patient with Kikuchi-Fujimoto disease accompanied with aseptic meningitis, which may be confused as a case of tuberculous meningitis and lymphadenitis.