OBJECTIVE To study the clinical features of histiocytic necrotizing lymphadenitis. METHODS Eleven patients with histiocytic necrotizing lymphadenitis were collected to analyze the clinical features, pathological features of biopsy lymph nodes, diagnosis and treatment. RESULTS Lymph node swelling of neck and persistent fever were detected in all patients. The leukocyte reduction and swiftness of ESR in serum were detected in most patients. The final diagnosis of histiocytic necrotizing lymphadenitis was confirmed by biopsy investigation of the pathology. The pathological features included distinctive necrosis, loss of lymph node structure, infiltration with histiocytes and lymphocytes, absence of neutrophils. All patients were treated with glucocorticoid for 2 to 4 months. The patients improved significantly and were not recurrence during follow up visit. CONCLUSIONS There was no specific clinical manifestation of histiocytic necrotizing lymphadenitis and which easy to misdiagnoses. Diagnosis of histiocytic necrotizing lymphadenitis relies on the pathological examination of enlarged lymph nodes.