Ectopic thoracic kidneys are rare anomalies, accounting for less than 5% of all renal ectopia. The anomaly is usually found incidentally on a chest radiograph, with no respiratory or systemic symptoms. It usually presents in the left posteroinferior thorax, associated with a congenital diaphragmatic defect or hernia and is more frequent in males than females. Associated anomalies of other organs are rare and inconsistent. We report a case of a 55-year-old female who was referred to our hospital for evaluation of a mass-like lesion in the right chest, identified incidentally on a chest radiograph. Contrast-enhanced chest computed tomography revealed a right ectopic thoracic kidney with a closed diaphragm and an accompanying pulmonary vascular malformation.