We report a case of lymphomatoid granulomatosis (LYG) with pharyngeal and pulmonary involvement. A 57-year-old man had developed skin ulcers on his leg five years ago and pharyngeal ulcers two years ago, which had regressed spontaneously. The patient had undergone antimicrobial therapy for bronchopneumonia one year ago. Almost two months later, he developed left recurrent laryngeal nerve palsy and was diagnosed with multiple cerebral infarctions associated with varicella-zoster virus vasculopathy and acute retinal necrosis from the infection due to the virus. During this period, chest computed tomography (CT) revealed multiple consolidations and ground-glass opacities that spontaneously disappeared within a week. He was admitted with multiple nodules and ground-glass opacities in both lung fields. A diagnosis of LYG was made from the histological findings of a computed tomography (CT)-guided lung biopsy. Additionally, LYG was reconfirmed from a past pathological specimen of the pharynx. LYG presented as various chest CT findings and multiple organ lesions. The radiological findings of this patient showed waxing and waning. Herein, we report the details of the case.