This case report describes the diagnosis and treatment of a 6-year-old child with hyperimmunoglobulin E syndrome (HIES) complicated by pulmonary artery pseudoaneurysm (PAPA). The child presented with hemoptysis, and chest computed tomography angiography (CTA) confirmed the diagnosis of PAPA. During interventional embolization, neither systemic arteriography nor main pulmonary artery angiography revealed the aneurysm; however, superselective angiography of the left lower pulmonary artery branch via a microcatheter successfully visualized the aneurysmal sac, which was then embolized with coils. Hemoptysis ceased after the procedure. The child underwent unrelated hematopoietic stem cell transplantation three months later and has remained free of hemoptysis during 18 months of follow-up, with imaging showing stable coil position and no recurrence of PAPA. This case highlights that in children with HIES presenting with hemoptysis, PAPA should be highly suspected. Interventional treatment requires combined systemic and multi-level pulmonary artery angiography for accurate localization, and active management of the underlying disease is crucial for preventing recurrence.