Rare case showing a relatively gradual progression of findings typical for Erdheim-Chester disease. The patient presented with renal obstruction due to ill-defined, infiltrative soft tissue in the renal hila and proximal ureters. 

Over the course of 9 years, the patient developed several more manifestations of the disease:

• increasing renal involvement, resulting in obstruction of the right kidney and typical "hairy kidney" appearance of the left
• adrenal infiltration
• biliary involvement with gallbladder and cystic duct thickening
• coating of the proximal great vessels and thoracic and abdominal aorta
• involvement and narrowing of the IVC
• infiltration of the RCA and right AV groove

Please see companion case with many overlapping imaging features.