Wiskott-Aldrich syndrome is an uncommon X-linked inherited disorder related to primary immunodeficiency, infections, eczema, and thrombocytopenia. A 21-year-old male patient with this syndrome underwent descending aortic aneurysm repair at the age of 12. The patient had ascending aortic aneurysm with aortic valve regurgitation and surgical aortic root replacement was performed. To the best of our knowledge, this is the first case with Wiskott-Aldrich syndrome operated due to aneurysms development in different segments of the thoracic aorta in both childhood and young adult periods.