Alone or in association with other lung or thorax component disorders, the airway wall (AWW) remains one of the most frequently involved elements in paediatric lung diseases. A myriad of AWW disorders will present with similar symptomatology. It is thus important for the clinician to reappraise the normal development and structure of the AWW to better understand the underlying disease patterns. We herein provide an overview of the structure of the AWW and a description of its development from the fetal period to adulthood. We also detail the most common AWW changes observed in several acute and chronic respiratory disorders as well as after cigarette smoke or chronic pollution exposure. We then describe the relationship between the AWW structure and lung function. In addition, we present the different ways of investigating the AWW structure, from biopsies and histological analyses to the most recent noninvasive airway (AW) imaging techniques. Understanding the pathophysiological processes involved in an individual patient will lead to the judicious choice of nonspecific or specific personalised treatments, in order to prevent irreversible AW damage.