First described in the 1970’s by Liebow et al., lymphomatoid granulomatosis (LYG) is currently defined by the World Health Organization Classification of Tumors as an extranodal angiocentric and angiodestructive lymphoproliferative disorder associated with infection by the Epstein-Barr virus (EBV) and/or immunodeficiency states. Lymphomatoid granulomatosis is more common in adults and has a preference for males. Patients are usually symptomatic and some may have concurrent extrapulmonary disease in the skin, central nervous system, and other anatomical sites. Lung lesions on chest radiographs and/or CT scans are nodular with or without cavitation. In some patients, the lesions may be reticulonodular. The gross anatomical features of LYG closely parallel the radiographic findings, consisting of nodular masses histologically composed of atypical B lymphoid cells permeating vascular walls with associated necrosis.