A 28-year-old man had a left pneumothorax and diffuse reticulonodular infiltrates. He subsequently had a right pneumothorax and manifested bilateral recurrences after treatment of the initial pneumothoraces. Pulmonary angiocentric immunoproliferative lesion was diagnosed by open lung biopsy. Treatment with combination chemotherapy improved the infiltrates, but failed to prevent another pneumothorax. Etiologic considerations for the recurrent pneumothoraces are proposed.