H syndrome is a rare autosomal recessive disorder characterized by a spectrum of clinical features, including hyperpigmentation, hypertrichosis, and multi-system involvement. Due to its rarity and the potential for systemic manifestations, the anesthetic management of patients with H syndrome presents unique challenges and requires a tailored approach. Facial and neck abnormalities, including sclerodermatous thickening and hypertrichosis makes airway management more challenging. Developing an individualized anesthetic plan based on the patient's specific H syndrome manifestations and comorbidities will help in successful management of these cases. Assessing the patient for comorbidities and potential organ involvement is essential as it might impact anesthesia and postoperative care due to multisystem involvement. This article aims to provide a comprehensive guide to anesthetic considerations and strategies for H syndrome patients. Herewith presenting a case of H syndrome in a 3 year old child with diagnosed H syndrome for Ludwigs angina for anesthesia management and airway management