Abstract
Pulmonary sclerosing pneumocytoma is a rare, typically asymptomatic, tumor with potential for malignant transformation. Diagnosis is frequently difficult due to its rarity and proclivity to mimic other pathologies. Here, we describe a particularly challenging case of a patient with a personal and familial history of other malignancies who presented with an incidental lung nodule, found to be pulmonary sclerosing pneumocytoma on pathologic examination after an anatomic lung resection. We conclude with a review of the natural history, clinical and histopathological presentation, and management options for this rare entity.