OBJECTIVE To investigate and analyze the clinical features in patients with histiocytic necrotizing lymphadenitis (HNL). METHODS A total of 68 HNL patients at our hospital between the years of 1999 to 2009 were enrolled. The clinical data were collected from the hospital records and the relevant literature was reviewed. RESULTS HNL mainly affected young people with an average age of (19 ± 13) years old and a female-to-male ratio of 1:1. All patients had lymphadenectasis. And 95.6% patients (65 cases) were feverish and 36.8% patients (25 cases) had mild hepatosplenomegaly; 25.0% (17 cases) upper respiratory symptoms such as sore throat and cough; 14.7% (10 cases) skin rash in their history; 51.5% (35 cases) leucopenia; 25.0% (17 cases)hepatic dysfunctions; 72.1% (44/61) elevated erythrocyte sedimentation rate (ESR); 11.1% (6/54) positive antinuclear antibody (ANA). The final diagnosis of HNL was confirmed by pathological examination and immunohistochemical staining of biopsy specimens. And 34 (50.0%) patients received glucocorticoid for 2 weeks to 3 months. Seven (10.3%) patients relapsed in which glucocorticoid was effective. Of 6 patients with positive ANA, one case was complicated with systemic lupus erythematosus (SLE) and another case diagnosed with SLE at 2 years after HNL. CONCLUSIONS The clinical manifestations of HNL lack specificity so that it can be easily misdiagnosed. While the etiology of HNL remains elusive, the histopathological examination of affected lymph nodes has contributed greatly to the final diagnoses of HNL. Glucocorticoid therapy is recommended for treatment. Generally, HNL has an excellent prognosis but it often relapses. It should be noted that HNL may coexist with SLE or evolve ultimately into SLE.