Purpose of Review <p>Fibrosing mediastinitis is an uncommon clinical pathologic syndrome driven by underlying infection and/or inflammation leading to structural and organ dysfunction from expansion of fibroinflammatory tissue. Overall, there is still scant literature summarizing the etiologies, progression, and management of fibrosing mediastinitis.</p> Recent Findings <p>Patients often present with unexplained shortness of breath and are found to have radiographic findings compatible with fibrosing mediastinitis. Although the leading cause in the United States is Histoplasmosis, there are various other infectious and inflammatory conditions that lead to fibrosing mediastinitis. The extent of fibrosis and organ involvement can be determined by chest radiographs or computed tomography. Therapeutic options include medications targeting the inflammatory cascade as well as surgical and non-surgical interventions on mediastinal vasculature, airways, and surrounding structures.</p> Summary <p>There are no unified treatment guidelines for fibrosing mediastinitis, so therapies must be tailored to each individual patient. Consideration should be made for early referral to a higher level of care if there is no multidisciplinary committee immediately available.</p>