| Summary: | <p>The histiocytosis is divided into Langerhans' cell histiocytosis (histiocytosis X) and non-Langerhans' cell histiocytosis (NLCH). The former mainly occurs in lymphatic hematopoietic tissue and often involves central nervous system (CNS). The latter includes Rosai-Dorfman disease (RDD), hemophagocytic lymphohistiocytosis (HLH), Erdheim-Chester disease (ECD), juvenile xanthogranuloma (JXG) and xanthoma disseminatum (XD), choroid plexus xanthogranuloma (CPXG), choroid plexus xanthoma (CPX), which may occur in lymphatic hematopoietic tissue and (or) CNS. This review has briefly introduced the clinicopathological characteristics and differential diagnosis of the above lesions. The incidence of these diseases is low in CNS, so they are often ignored, misdiagnosed and impertinently treated by neurosurgeons and pathologists. Therefore, understanding their clinical and pathological characteristics is conducive to improve the diagnosis and therapy.</p><p> </p><p><strong>DOI: </strong>10.3969/j.issn.1672-6731.2015.04.002</p>
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