Abstract: Castleman disease (CD) refers to a group of uncommon lymph node proliferative disorders that have highly clinically heterogeneous and obvious pathological features. The etiology and pathogenesis of CD remain unclear. The pathogenesis of unicentric CD (UCD) is most likely driven by clonal proliferation and acquired mutations of tumor stromal cells. Idiopathic multicentric CD (iMCD) intersects with a variety of diseases in terms of its clinical manifestation and pathological features including autoimmune factors, paraneoplastic syndrome, viral infectious factors and inflammatory factor disorders. The pathogenesis of iMCD is probably the common pathway of immune dysregulation and cytokine increase caused by the combined action of multiple etiologies. Here, we aim to summarize the latest reported etiology and pathogenesis of CD, aiming to deepen the understanding of this disease.