Recurrent pericarditis is a true challenge for clinicians, especially when the patient becomes unresponsive or not tolerant to conventional treatments. An accurate diagnosis of recurrent pericarditis, possibly supported by advanced imaging tools, is critical to provide timely and appropriate treatment of symptoms and prevention of further episodes. The incessant research on the inflammatory pathways underlying cardiovascular diseases, led recently to the assessment of anti interleukin-1 agents in the setting of recurrent pericarditis. This review will focus on the diagnostic assessment of recurrent pericarditis, along with the most modern therapeutic advances in this field. Bibliographic databases were searched (MEDLINE/PubMed, BioMed Central, the Cochrane Collaboration Database of Randomized Trials, Scopus, ClinicalTrials.gov, EMBASE, Google Scholar) using the terms "recurrent pericarditis" AND "diagnosis" OR "treatment" OR "IL-1" OR "inflammation".