Objective: As a rare cause of fever and lymphadenopathy (especially in the neck) in children, the purpose of this study is to explore the clinical characteristics of histiocytic necrotizing lymphadenitis (HNL) in children and improve the diagnosis and treatment of this disease. Methods: We retrospectively collected the data of 6 pediatric patients with HNL diagnosed by lymph node biopsy from 2012 to 2021 in department of pediatric, the affiliated Changsha Central Hospital, Hengyang Medical School, University of South China, including the clinical characteristics, laboratory examinations, imaging findings and histopathological examinations, treatment and follow-up of the patients and reviewed relevant literature. Results: All the patients were Han girls, with no kinship with each other, and four cases were from rural areas. The age of onset was from 7 to 14 years old, with durations ranged from 1 week to 3 months. All children had cervical lymphadenopathy with tenderness, five cases of fever, two cases of cough, one case of weight loss, four cases of leukopenia, one case of liver impairment, one case of combined bacterial infection of leukocytosis. All children underwent lymph node biopsy and were diagnosed with HNL, four children were treated with anti-infection before diagnosis, two children were treated with steroid therapy after diagnosis, and 1 case of weakened immune function was used immunoglobulin for modulating immunity. All children improved and discharged, and no recurrence was observed until October 2021. Conclusion: In our study, the children with swollen lymph nodes in the neck were accompanied by tenderness and fever as the main clinical manifestations, without specificity. Histopathology and immunohistochemical examination by removing the affected lymph nodes could confirm HNL. Most children had a good prognosis and long-term follow-up were required.