A 33-year-old woman with a family history of multiple cancers had been undergoing surveillance scans. Magnetic resonance imaging revealed cysts in the kidney and pancreas, multiple hemangiomas in the liver, and a lesion in the left lower lobe (LLL) of the lung. Repeat positron emission tomogram/computed tomography demonstrated a 1.8 1.6-cm nodule (Fig. 1) within the LLL. The patient underwent endobronchial ultrasound– guided fine needle aspiration biopsy of the LLL nodule, which showed hypercellular smears containing uniform, cuboidal, round, and oval medium-sized cells. They were diffusely positive for thyroid transcription factor-1, and negative for neuroendocrine cell markers. The diagnosis was well-differentiated lung adenocarcinoma. The patient underwent video-assisted thoracoscopic LLL lobectomy, which showed a well-circumscribed 2.5-cm yellow-tan nodule composed of mostly monomorphic, cuboidal surface cells and round cells with pink to clear cytoplasm and well-defined cell borders in papillary, solid, and sclerotic growth patterns. Occasional hemorrhagic foci were also present. A focal area of mild cytologic atypia was noted. A focus of metastatic tumor deposit containing solid sheets of cells was identified in one of the peribronchial lymph nodes. The results of staining for thyroid transcription factor-1 and epithelial membrane antigen were positive in both surface and round cells, but cytokeratin 7 and cytokeratin AE1/AE3 stained only surface cells (Fig. 2). The cytomorphologic examination findings and immunoprofile were consistent with sclerosing pneumocytoma (SP) with a lymph node metastasis. SP (previously called sclerosing hemangioma) is a rare tumor of the lung that is thought to arise from primitive respiratory epithelium. This tumor usually occurs in middle-aged adults with a male-to-female ratio of 1:5. 1 Surgical resection is curative without a need for additional treatment. Although SP is considered a benign tumor, we found a lymph node (LN)