Autoinflammatory diseases are rare, genetically driven disorders marked by dysregulated innate immune responses, leading to episodes of systemic inflammation without infection or autoantibodies. Conditions such as familial Mediterranean fever, cryopyrin-associated periodic syndromes and TNF receptor-associated periodic syndrome manifest with recurrent fevers, rash, arthritis, serositis and organ inflammation. Due to their rarity and clinical overlap with autoimmune diseases, infections and other inflammatory conditions, autoinflammatory diseases are often misdiagnosed or inappropriately managed. Misdiagnosis as autoimmune diseases can delay appropriate treatment, highlighting the importance of recognising hallmark features such as episodic fevers, serositis and negative autoimmune markers. Additionally, the misinterpretation of a febrile patient with accompanying abdominal pain as having an intra-abdominal inflammatory condition may lead to unnecessary surgical intervention. Genetic testing is critical for confirming diagnoses but is underutilised. Mismanagement with broad immunosuppressants may suppress symptoms but fail to address underlying mechanisms, while targeted therapies, including IL-1 and IL-6 inhibitors, provide optimal outcomes. Delayed treatment of complications like amyloidosis underscores the need for early and aggressive inflammation control. In most cases, patient education is essential to improve disease outcomes.