Histiocytic necrotizing lymphadenitis (HNL) is a kind of rare benign disease, predilection to involve the neck of young women, causing enlargement of the lymph nodes and usually accompanying by fever and other constitutional symptoms, and a unique clinicopathologic course are characteristic of this disease. Because its histomorphology has a given spectrum changing, it may lead to misdiagnosis for someone who is unfamiliar with it. According to the document records, misdiagnostis of HNL could be up to 30%-40%, especially in its early proliferative stage. In this 31 cases analysis, the authors systematically summarize the clinicopathologic characteristics of this lesion, and focus to elucidate the different features between HNL and lymphoma. Although presently it is thought that the disease is self-limited, there are two cases with clinical manifestations of systemic lupus erythematosus in our series. Possibly, the two diseases may have some intrinsic relationships. Long term follow-up of its final outcome in these patients is necessary to clarify whether or not HNL is a self-limiting or progressive disease.