A 74-year-old male visited the emergency department for a few days of dyspepsia and abdominal pain unresponsive to digestive medicine. He was febrile, and laboratory tests had following results: white blood cells, 16210/µL; blood urea nitrogen, 62.8 mg/dL; serum creatinine, 4.30 mg/dL; alkaline phosphatase, 887 U/L; direct bilirubin, 1.47 mg/dL; gamma glutamyl transferase, 165 IU/L; amylase, 1115 IU/L; lipase, 1161 IU/L; C-reactive protein, 176.97 mg/L. Although he did not show any pulmonary symptoms, computed tomography (CT) showed only edematous changes around the common bile duct (CBD) (Fig. 1A) and reticular opacity showing a suspicious pattern of interstitial lung disease or collagen vascular disease in both lower lobes (Fig. 1B). Magnetic resonance cholangiopancreatography (MRCP) showed CBD dilatation and tiny renal cysts (Fig. 1C). His symptoms improved after antibiotics and endoscopic nasobiliary drainage. Despite appropriate therapy, serum creatinine increased to 8.50 mg/dL after two weeks, and other laboratory results were as follows: c-anti-neutrophil cytoplasmic antibody (ANCA) and p-ANCA, 1521.95 and 14.48 AAU; antinuclear antibody (ANA), negative. We initiated hemodialysis for control of uremic symptoms, and performed a renal biopsy. The pathologic results showed granuloma formation with Langerhans giant cells and fibrous crescent (Fig. 2A). Granulomatosis with polyangiitis (GPA) was diagnosed. We performed high dose-steroids and cyclophosphamide treatments concurrently with hemodialysis. Two months later, follow-up magnetic resonance imaging (MRI) was performed to identify the CBD. Interestingly, as compared to the previous MRCP, contrast-enhanced nodular lesions with increased number and size were seen in the kidneys (Fig. 2B), and a contrast-enhanced lesion was seen in the pancreatic head around CBD (Fig. 2C). The nodular lesions on the kidney turned out to be arterial aneurysms (Fig. 2D). The immunosuppressive agents and hemodialysis were continued, but pneumonia developed, and progressed to sepsis and the patient died. GPA is a systemic vasculitis characterized as necrotizing granulomatous inflammation. GPA can involve systemic organs.1 GPA mainly presents as a phenotype of rapidly progressive glomerulonephritis in glomeruli.2 However, as GPA of relatively long duration can lead to medium-sized arterial aneurysms.3 Acute cholangitis might result from the pancreatic pseudoaneurysm around CBD. The pancreatic pseudoaneurysm in follow-up, contrast-enhanced MRI may be caused by chronic GPA in this patient. Clinically, this involvement of GPA may develop various manifestations. Therefore, if the patient is clinically suspected of having a systemic vasculitis, such as GPA, nephrologists or clinicians should consider whether clinical symptoms or signs of GPA can be manifested in various ways.