Background: Hyperesinophilia (HES)with Hereditary angioedema (HAE), is a rare disease characterized by peripheral blood eosinophilia > 1500/mcl (> 1.5 × 109/L) persisting ≥ 6 months and the presence of end-organ damage. Hereditary angioedema is a lifelong illness characterized by recurrent swelling of the skin, intestinal tract, and, the upper airway. It results from insufficient activity of the C1-inhibitor protein, leading to disturbances in the kallikrein/bradykinin pathway. Case presentation: We reviewed a case of Hypereosinophilia with hereditary angioedema (HAE) in 7-year-old boy who was referred from another hospital in Coimbatore for further management. He had elevated absolute eosinophilic count & elevated IgE. Bone marrow aspiration and biopsy were done which showed increased eosinophil. Stool for ova/ cyst was negative. He was initiated on oral prednisolone (10mg), pantaprozole (20mg) & dexamethasone (50mg). Conclusion: Hereditary angioedema (HAE) is a genetic condition that poses a threat to life; this condition necessitates rapid diagnosis and treatment to control acute attacks and avert potentially lethal complications, especially when swelling impacts the airway, and can despite recent developments in treatment options, a child quality of life can be significantly affected.