Recurrent pericarditis poses a significant challenge to clinicians, particularly when patients are unresponsive or intolerant to conventional treatments. Accurate diagnosis of recurrent pericarditis, potentially facilitated by collaboration with other medical specialties, is crucial for ensuring timely and appropriate treatment of symptoms and prevention of further episodes. We present a case of a 52-year-old male patient with a history of multiple episodes of pericarditis, who was admitted to the Cardiology Department due to another recurrence. The first episode of pericarditis was diagnosed nearly a year before his current hospitalization. Initially, the patient received high doses of Ibuprofen and colchicine, but there was no favorable response to this treatment regimen. At that point, intravenous prednisolone was initiated, which led to clinical and laboratory improvement. Since then, the patient had experienced two more recurrences while tapering off prednisolone. Immunological tests, Mantoux tuberculin skin test, and chest and abdominal computed tomography (CT) had revealed no evidence of an underlying cause. On admission the patient was febrile and the electrocardiogram showed diffuse ST elevation and PR depression in leads I, II, aVF, V2-V6. Bedside echocardiogram revealed a small pericardial effusion and since the chest X-ray was normal and no other potential infection sites were identified, the diagnosis of recurrent pericarditis was established. During his current hospitalization, intravenous prednisolone was initiated, colchicine was continued and a more detailed history was taken, raising the suspicion upon the presence of an auto-inflammatory disease. Genetic investigation identified an uncommon heterozygous mutation in the familial Mediterranean fever gene (MEFV) and after consideration of patient's history, familial Mediterranean fever was diagnosed. Anakinra was initiated on top of colchicine and gradual tapering of corticosteroids and the patient showed significant improvement, with no other recurrence during the two-year follow-up.