Idiopathic focal fibrosing mediastinitis with unilateral pulmonary arterial and venous involvement

We report a case of a 1-year-old girl where computed tomography (CT) angiography demonstrated presence of ill-defined heterogenous soft tissue thickening obliterating the mediastinal fat planes in right peri-hilar region and encasing the hilar right pulmonary artery and its segmental branches leading to marked narrowing with nonvisualization of the right-sided pulmonary veins. The case highlights the features of fibrosing mediastinitis as well as the role of CT angiography in the diagnosis and accurate depiction of the extent of involvement of the mediastinal vasculature and airways.