IgG4-related disease (IgG4-RD) is an immune-mediated¹ fibro-inflammatory condition^1-3 that can involve multiple organ systems^1-2. Diagnosis criteria are based on clinical, serological, radiological, histopathological features and response to corticosteroid therapy^{1-2, 4-7}.

Obscure clinical presentations, diverse spectrum of imaging manifestations, overlapping features with infectious and neoplastic conditions^{1-2, 6-8} frequently constitute diagnostic challenges and potential delay in diagnosis.

High index of suspicion and awareness among radiologists is critical in achieving accurate and timely diagnosis, facilitating early initiation of immunosuppressive therapy^{1-2, 9}, and therefore prevention of irreversible tissue fibrosis^{1, 3, 9} and organ damage⁹.