<p>Kimura disease (KD) is a rare inflammatory condition with an unclear etiology (Mai in Front Pediatr 11:1131963, 2023). Diagnosing Kimura disease is highly dependent on the clinical presentation of head and neck subcutaneous swelling, with parotid gland swelling being the most common (Gupta in Indian J Otolaryngol Head Neck Surg 71:855–859, 2019), in addition to peripheral eosinophilia and an elevated serum immunoglobulin E level (Lee et al. in Front Med 9:1069102, 2022). Cervical lymphadenopathy is a common finding at the initial presentation, and histopathological examination is usually supportive with lymphoid follicular hyperplasia and eosinophilic infiltration (Gupta in Indian J Otolaryngol Head Neck Surg 71:855–859, 2019). While KD is rare and usually benign, the diagnosis is often late, and that might lead to unnecessary investigations and delayed management (Mai in Front Pediatr 11:1131963, 2023). As Th2 inflammatory pathway activation and release of cytokines like IL-4, IL-5, and IL-13 has been found in patients with Kimura disease, a targeted therapy blocking one of these pathways is intriguing (Shang et al. in Immun Inflamm Dis 11:e1084, 2023). Dupilumab has been used in patients with Kimura disease and has shown great efficacy, including in one pediatric case (Shang et al. in Immun Inflamm Dis 11:e1084, 2023). We treated our presented patient with Dupilumab, and his clinical status and laboratory values showed a great response with no major side effects. Here, we present the youngest reported patient receiving Dupilumab treatment for Kimura disease, showing an excellent response and adding to the existing scarce literature on this topic.</p>