Fibrosing mediastinitis is an uncommon and intriguing complication of histoplasmosis. This condition is characterized by paucicellular, proliferative fibrosis within the mediastinum that distorts, encompasses, or obliterates adjacent anatomic structures. Its pathogenesis remains elusive but may reflect an unabated immune response to Histoplasma antigens; an association between the major histocompatibility complex class I molecule human leukocyte antigen-A2 and fibrosing mediastinitis has been reported. Additionally, there is no evidence that mediastinal lymphadenitis or mediastinal granuloma evolves into fibrosing mediastinitis. Symptoms originate from impingement on adjacent structures by fibrosis. Patients can present with cough, dyspnea, and hemoptysis related to airway and/or vascular involvement. Esophageal involvement can result in dysphagia, reflux, and traction diverticulum. In endemic areas, diagnosis is based on clinical suspicion given its unique radiographic features and biopsy is often unnecessary. Two distinct radiographic patterns have been observed: local, calcified changes (often right paratracheal) and a diffuse, noncalcified form. The localized, and often calcified, variant seems to be more commonly associated with posthistoplasmosis fibrosing mediastinitis; the diffuse form is less common and can be associated with other conditions such as retroperitoneal fibrosis and methysergide therapy. Unilateral pulmonary edema and pleural effusion has been recognized as a complication of fibrosing mediastinitis with pulmonary venous occlusion. The “pseudomitral stenosis” syndrome, which can be seen in fibrosing mediastinitis, is characterized by dyspnea and pulmonary venous occlusion and can lead to hemoptysis and pulmonary hypertension.