RESUMEN
Immunoglobulin G4 (IgG4)-related disease is a chronic systemic disease. It is characterized by inflammatory fibrosis and high serum IgG4 levels. IgG4-positive plasma cells infiltrate target organs in this disease. It may involve the pancreas, biliary tract, lacrimal glands, salivary glands, orbits, thyroid, kidneys, lymph nodes, or retroperitoneum. It may present as vasculitis with involvement of large to medium sized vessels such as the aorta, the common iliac, carotid, and coronary arteries. We present a case of 55-year-old male patient who presented with shortness of breath on exertion and atypical chest pain. On CT angiography, a giant coronary artery aneurysm involving the left anterior descending artery, multiple visceral and intercostal artery aneurysms, and nodular paravertebral soft-tissue thickening secondary to IgG4 vasculopathy.