Idiopathic multicentric Castleman disease(iMCD) is a rare and highly heterogeneous lymphoproliferative disorder, which represents a distinct subtype of Castleman disease(CD). In 2018, it was included in the China's First List of Rare Disease issued by the National Health Commission of the People's Republic of China. The prognosis of iMCD patients remains poor, with a previously reported 5-year overall survival rate ranging from only 51% to 77%. This highlights an urgent need for effective treatments. The successful application of interleukin-6(IL-6) targeted therapies represents a major breakthrough in the treatment of iMCD. However, a considerable number of patients still fail to benefit from IL-6 targeted treatments. With in-depth research into the molecular mechanisms of the disease, non-IL-6 targeted therapies have gained increasing attention. Conventional regimens targeting plasma cell disorders or lymphoma-like conditions such as anti-CD20 monoclonal antibodies, bortezomib-cyclophosphamide-dexamethasone, and thalidomide-cyclophos-phamide-prednisone, as well as emerging therapies, including mTOR pathway inhibitors, Bruton's tyrosine kinase inhibitors, JAK-STAT pathway inhibitors, and tumor necrosis factor inhibitors, have shown promising efficacy in iMCD treatment.These developments contribute to the progress in iMCD therapy.This article will elaborate on the therapeutic progress of iMCD, with the aim of providing reference for clinical practice.