We review here a 50-year-old male with a history of intramural hematoma of the aortic arch. An increase in lesion size and thickening of tissue bands encircling the ascending aorta, the aortic arch, and the abdominal aorta were observed while considering the differential diagnosis of lymphoproliferative syndrome. Imaging with 18 F-FDG PET/CT revealed an abnormal tracer uptake surrounding the retroperitoneal and mediastinal aorta (including the aortic arch), which suggested the possibility of fibrosis of the entire aorta. A biopsy was taken that turned out to be compatible with a fibrotic lesion. Retroperitoneal fibrosis is a rare disease characterized by retroperitoneal tissue that could be included under the definition of chronic periaortitis [1, 2]. Men aged 50–60 years old are affected two to three times more often than women. The most common symptoms include truncal pain and systemic inflammatory symptoms [3]. 18 F-FDG PET/CT is useful for evaluating the metabolic activity of the mass, revealing other diseased sites, and detecting secondary or associated occult neoplastic or infection processes [4, 5].