A 14-year-old male patient presented with a nonproductive cough, weight loss, fatigue, and malaise. A chest radiograph showed large bilateral cavitary lung lesions in both upper and lower lobes that failed to improve with antibiotics and anti-inflammatory medications. Infectious and rheumatologic work-ups were negative. Thoracoscopic lung biopsies were diagnostic for nodular lymphocyte predominant Hodgkin lymphoma (NLPHL). The patient received combination chemotherapy and immunotherapy based on current treatment standards with an excellent clinical response. NLPHL is a rare B-cell lymphoma that typically presents as peripheral lymph nodal disease, clinically distinct from classical Hodgkin lymphoma. The prognosis of NLPHL in children is favorable, although relapse rates are high. This case details several unique features of NLPHL and describes the presentation, diagnosis, and treatment of an adolescent male with a rare pulmonary and cervical NLPHL, the first such case described in a pediatric patient.