Castleman disease (CD) is divided clinically into unicentric or multicentric type. Pathologically, CD is divided into hyaline-vascular and plasma cell variants. Unicentric CD is most common, about 75% of these cases are hyaline-vascular variant, and surgical excision is often curative. In contrast, there are a number of types of multicentric CD including HHV8-associated, idiopathic, and a subset of cases that arise in association with POEMS syndrome. Therapy is required for most patients with multicentric CD, but there is no consensus approach currently. As is evidence, the designation Castleman disease encompasses a heterogeneous group of diseases of varied pathogenesis and which require different therapies.