Case reportInferior Vena Cava agenesis presenting as deep vein thrombosis in an eight year-old girlMentesidou, Lidaa; Dettoraki, Athinab; Michalopoulou, Aikaterinib; Pergantou, Helenb; Malama, Asteroc; Gavra, Mariac; Bachou, Theodoraa Author Information a1st Department of Pediatrics, ‘Panagiotis and Aglaia Kiriakou’ Children's Hospital bHaemophilia Centre/Haemostasis and Thrombosis Unit cDepartment of Radiology, ‘Aghia Sophia’ Children's Hospital, Athens, Greece Correspondence to Lida Mentesidou, 1st Department of Pediatrics, ‘Panagiotis and Aglaia Kiriakou’ Children's Hospital, Thivon 1 and Levadias, Goudi, Athens 11527, Greece. Tel: +302132009355; e-mail: [email protected] Received 22 May, 2022 Revised 11 September, 2022 Accepted 25 October, 2022 Blood Coagulation & Fibrinolysis: November 11, 2022 - Volume - Issue - 10.1097/MBC.0000000000001178 doi: 10.1097/MBC.0000000000001178 Buy PAP Metrics Abstract Agenesis of vena cava inferior (AVCI) is a rare congenital malformation with a prevalence of 0.0005–1% in the general population. High level of suspicion is required in young patients with deep vein thrombosis (DVT), particularly bilateral. We present an 8-year-old girl with AVCI presenting as bilateral lower extremity DVT and a review of the literature in pediatric cases with AVCI and DVT. Copyright © 2022 Wolters Kluwer Health, Inc. All rights reserved.