A 34-year-old man presented with a severe headache, fever, and cervical lymphadenopathy followed by generalized tonic-clonic seizure. Evaluations showed splenomegaly, elevated liver enzymes, and 380 white blood cells/mm3 in the cerebrospinal fluid. Two weeks after admission, he developed sudden vertigo. Examination revealed spontaneous horizontal-torsional nystagmus to the right and bithermal caloric tests documented left canal paresis. A cervical lymph node biopsy disclosed subacute necrotizing lymphadenitis. We report a case of aseptic meningitis and unilateral vestibulopathy associated with histiocytic necrotizing lymphadenitis (Kikuchi