Idiopathic Multicentric Castleman Disease (iMCD), a disease of unknown etiology is characterized by angiofollicular hyperplasia. Many of its features, attributed to the overactivity of interleukin 6 (IL-6), can be seen in other conditions such as diseases of inflammatory, infectious or neoplastic nature. Until the introduction of the diagnostic guidelines for iMCD, the reported literature included numerous cases of seemingly mixed presentation. To avoid misdiagnoses, diagnostic criteria was approved in March 2017 by experts from different fields including hematology, oncology, and rheumatology. The diagnosis of iMCD requires the exclusion of multiple conditions that can present in a similar manner. However, the process of exclusion is not always straightforward. We carried out a literature review to gauge the impact of the new criteria with respect to diagnostic accuracy, exclusion of mimicking conditions and reported difficulties in using the above-mentioned criteria. We also reviewed the latest trends in the treatment of iMCD using the collected data. A total of 39 cases were identified. 76% of the cases (n=30) reported successful application of the full diagnostic criteria. Only 24% (n=9) cases were reported with overlapping conditions suggesting unsuccessful use of exclusion criteria. Tocilizumab was the most commonly used therapeutic agent in 46.1% (n=18) of the cases followed by rituximab, sirolimus, and siltuximab. The majority of the physicians diagnosed iMCD accurately and described minimal to no difficulties in applying the diagnostic criteria. Also, the majority of the patients responded well to therapies targeting IL-6 in addition to other immunomodulatory/immunosuppressive agents.