Isolated endobronchial metastasis from extrapulmonary solid organ was rarely reported in previous literature. We report an isolated endobronchial metastasis of Wilms' tumor in a 4-year-old boy. He underwent right nephrectomy on account of Wilms' tumor at the age of 3 years. We performed a wedge resection of a metastasis to the right lower lobe of the lung a year later, followed by chemotherapy and irradiation of the involved lung over a period of 30 weeks. One and a half years later, he developed obstructive pneumonitis of the left lung. Fiberoptic bronchoscopy identified a tumor at the left main bronchus with near total bronchial obstruction, compatible with findings on a computed tomographic scan. Rigid bronchoscopy was performed to core out the obstructing tumor. It was reported as a metastatic Wilms' tumor. Successful reexpansion of the left upper lobe was achieved and he was discharged uneventfully, followed by adjuvant chemotherapy.
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