Background: Turner syndrome (TS) is a rare genetic condition characterized by complete or partial X-chromosome monosomy. It is associated with distinct phenotypic features, most notably webbed neck (pterygium colli) and ocular abnormalities such as medial epicanthus. Surgical correction of these conditions is technically challenging and associated with high recurrence rates, particularly in the pediatric population.

Case Report: We report the case of a 9-year-old girl with TS and multiple comorbidities, including congenital heart disease and a history of sequential surgical interventions since age 4. The patient underwent cervical and ocular reconstruction through serial Z-plasties and rotation flaps.

Discussion: The 5-year longitudinal follow-up demonstrates that a staged surgical approach, integrated with multidisciplinary care (endocrinology and cardiology), is essential to manage growth-related changes and systemic risks.

Conclusion: Long-term monitoring highlights the importance of individualized surgical planning and the adaptation of techniques throughout the patient's development to optimize functional and aesthetic outcomes, while mitigating the psychosocial impact of the syndrome.