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Wolf-Hirschhorn syndrome (WHS) is an uncommon genetic disorder that often presents with characteristic facial features, developmental delay and potential airway anomalies, which can complicate the anaesthetic management. We present a case of WHS in early childhood who came with severe upper airway obstruction due to grade 4 adenotonsillar hypertrophy and required an emergency tracheostomy. The situation was made even more challenging by craniofacial abnormalities, a submucosal cleft palate and a history of laryngomalacia. Anaesthesia was induced with inhalational sevoflurane and intravenous ketamine to preserve spontaneous ventilation. A multidisciplinary team approach, along with preparation for a difficult airway, ensured safe tracheostomy with minimal complications. This case highlights the importance of thorough preoperative assessment, preservation of spontaneous ventilation, preparedness for airway emergencies, with close communication with the surgical team during the perioperative period. It also emphasises the need for heightened vigilance in managing rare syndromes with potential multisystem involvement.