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We report a case of a massive primary sclerosing pneumocytoma (PSP) involving the right lower lobe adhering esophagus with small synchronous PSP on the superior segment of the left lower lobe with concurrent mutation for B-RAF proto-oncogene, serine/threonine kinase (BRAF V600E), and phosphatase and tensin homolog (PTEN) gene in a young female. She underwent right lower lobectomy and mediastinal lymph node dissection under single lung ventilation with tumor-free margins on diagnosis-based findings of preoperative computed tomography-guided
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