Hyperimmunoglobulin E syndrome (HIES) is an immunodeficiency disorder that manifests itself by affecting more than one system. Arterial aneurysms are among the significant complications associated with HIES. Surgical procedures for patients with such aneurysms are uncommon. In this study, we aim to present the case and surgical repair of a male child who was previously diagnosed with HIES and presented with rapidly expanding ascending aortic aneurysm.
