National Research Institute of Tuberculosis and Lung Disease (NRITLD), Shahid Beheshti University of Medical Sciences, Tehran, Iran TANAFFOS (Respiration) 1735-0344 20 3 2021 07 01 Ectopic Intrathoracic Kidney Associated with Ipsilateral Ectopic Spleen and Diaphragmatic Hernia in a Pediatric Patient: A Case Report 291 293 251200 EN Mitra Naseri Department of Pediatric Nephrology, Faculty of Medicine, Mashhad University of Medical Sciences, Mashhad, Iran Seyyedeh Fatemeh Ghalibafan Faculty of Medicine, Mashhad University of Medical Sciences, Mashhad, Iran Seyed Ali Alamdaran Department of Pediatric Radiology, Faculty of Medicine, Mashhad University of Medical Sciences, Mashhad, Iran Journal Article 2022 03 16 <strong>Background: </strong>Intrathoracic kidney is the rarest form of an ectopic kidney that is usually accompanied by left congenital diaphragmatic hernia (CDH) (Bochdalek hernia), the association of which with other anomalies is rare. <strong>Case Presentation:</strong> Herein, we describe a case with a diagnosis of an intrathoracic kidney associated with the ectopic spleen and diaphragmatic hernia diagnosed during imaging studies for urinary tract infections (UTIs). This study reports an 11-month-old male case with a history of CDH and several episodes of UTIs. A kidney ultrasound revealed that the left kidney and spleen were located in the thoracic cavity. Despite intrathoracic lying of the left kidney, there was no vesicoureteral reflux. Technetium-99m dimercaptosuccinic acid scan reported a highly positioned left kidney.  <strong>Conclusion:</strong> With the consideration of a pediatric literature review among patients with intrathoracic ectopic kidney, our case was special and notable since it was the first neonate who had an association of intrathoracic spleen and kidney in the same side with a delayed diagnosis. The main point of this case was that radiologists should consider thoracic kidney a differential diagnosis of unilateral renal agenesis when there is a history of diaphragmatic hernia. https://www.tanaffosjournal.ir/article_251200_bfb3206155832047330e55a331d6734e.pdf