Background <p>Necrotizing tonsillitis is rare and may lead to life-threatening upper airway obstruction in children, requiring emergency airway management.</p> Case presentation <p>An 8-month-old boy presented with unresolved fever and was diagnosed with acute tonsillitis. Despite prior treatment with amoxicillin and paracetamol, the fever persisted, accompanied by leukopenia. Intravenous C-penicillin was initiated, but respiratory distress ensued, necessitating non-invasive ventilatory support and subsequent intubation due to increased stridor. Intubation was challenging due to copious secretions and a floppy epiglottis, but successful intubation was achieved on the second attempt using a C-MAC^® video laryngoscope with a Miller blade size 0. Computed tomography (CT) revealed a large collection with mucosal involvement in the peritonsillar and tonsillar regions, extending to adjacent structures. Direct laryngoscopy, tissue sampling, and multiple surgical debridements were performed as the patient’s condition deteriorated. Perianal excoriation and diarrhea raised suspicion of primary immunodeficiency syndrome. <i>Pseudomonas aeruginosa</i> and <i>Stenotrophomonas maltophilia</i> were isolated from tissue cultures and effectively treated with targeted antibiotics. Serological testing showed positive IgG for herpes simplex virus 1 (HSV-1), while immune deficiency testing indicated a normal immune status, pending genetic testing results. After 21 days of ventilation, the patient was extubated, received non-invasive ventilatory support, and was discharged with oral antibiotics.</p> Conclusion <p>This case highlights the critical nature of necrotizing tonsillitis, especially in infants with suspected primary immunosuppressive disorders. Early recognition, prompt airway management, and surgical intervention are crucial for optimal outcomes.</p>