Introduction: The intrapulmonary involvement of Castleman disease is extremely rare with very few cases reported in the literature. Case report: We present a case of a 16-year-old male who underwent pulmonary resection for Castleman disease. The tumor was located at the right hilum and measured 10 cm in diameter. The patient underwent an intrapericardial pneumonectomy with a mediastinal lymph node dissection because the tumor showed infiltration into the lung parenchyma, the major pulmonary vessels and the main bronchus. Conclusion: We emphasize that some cases with intrapulmonary Castleman disease may require a pneumonectomy if the tumor is centrally located.