Immunoglobulin-G4-related disease (IgG4-RD) is a fibro-inflammatory condition that can impact any organs/tissues, including the vascular systems, resulting in aortitis/periaortitis/periarteritis (PAO/PA). The complex nature of this disease and limited understanding have led to potential delays in identifying and managing irreversible organ damage. Herein, we report a 17-year-old female with hyper IgG4 disease, sclerosing mesenteritis, short stature, and insulin resistance who presented with symptoms of fever, epigastric pain, left flank pain, vomiting, dizziness, decreased urine output, and diarrhea. Imaging studies revealed an arterial wall thickening of the ascending aorta and aortic arch, splenic abscesses, and enlarged lymph nodes, consistent with IgG4-related aortitis. Treatment with steroids and antifungal agents was initiated. However, the patient developed septic shock and multi-organ failure requiring inotropes and mechanical ventilation. Ascending aortic aneurysm rupture, in this case, probably led to the patient's demise, but unfortunately, no autopsy was done to confirm it. This case highlights the importance of identifying and addressing vascular involvement in IgG4-RD to prevent irreversible organ damage and mortality.