Abstract: Castleman disease (CD) is a rare lymphoproliferative disease that is clinically classified into unicentric Castleman disease (UCD) and multicentric Castleman disease (MCD). According to the status of human herpes virus-8 (HHV-8) infection, MCD is further classified into HHV-8-positive MCD, and HHV-8-negative MCD, which is also called idiopathic multicentric Castleman disease (iMCD). There are standard treatment options for both UCD and HHV-8-positive MCD, but there has been no uniform standard of diagnosis and treatment for iMCD, which mainly relied on the experience of clinicians. In recent years, Castleman Disease Collaborative Network (CDCN) has newly defined the concept and diagnostic criteria of iMCD, and a comprehensive guidance on the treatment of iMCD was established in November 2018. In this paper, we try to review the current treatment options and advances of iMCD, which might help Chinese clinicians to diagnose and treat this rare disease.