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Introduction: Arterial tortuosity syndrome (ATS) is an extremely rare autosomal recessive disorder of the connective tissue. It is characterized by tortuosity and elongation of medium and large arteries, with multiple disorders associated with the widespread involvement of the connective tissue., Case Report: Newborn diagnosed with ATS, with multiple vascular malformations, hiatal hernia, and bilateral inguinal hernia. He underwent surgery at three months of age. The hiatal hernia was closed, and bilateral inguinal hernia repair was performed. The inguinal hernias required up to 4 surgeries as a result of recurrences.During follow-up, the patient had retrocardiac diaphragmatic hernia. It was operated on, with subsequent incisional hernia. 8 years later, he was admitted as a result of septic shock secondary to intestinal occlusion. Emergency surgery was scheduled, demonstrating gastric herniation in the right pleural cavity, with perforation of the fundus. The patient died at the ICU 24 hours later., Discussion: The pediatric surgeon should be familiar with ATS, since it may cause multiple surgical pathologies, it is difficult to manage, and it is associated with a high risk of recurrence and complications.