Summary Eleven cases of histiocytic necrotizing lymphadenitis were studied. Originally four were misdiagnosed as cervical tuberculous lymphadenitis. The characteristic symptoms and signs were local tenderness, fever, leucocytopenia and good prognosis. Antibody to Epstein-Barr virus was elevated in 2 of 3 cases. Biopsy of the affected lymph node was necessary when the differential diagnosis from lymph node tuberculosis or malignant lymphoma could not be made. The characteristic histological feature was focal necrosis without neutrophil infiltration in cortical or subcortical areas. Ultrastructurally, we found characteristic inclusions (‘tubulo-reticular structures’) in histiocytes and lymphocytes.