Granulomatosis with polyangiitis (GPA) is a small to medium vessel vasculitis that is uncommon in paediatrics. However, with chronic cough often being the initial symptom, a common complaint and a median age of diagnosis of 14 years, it is nevertheless an important condition for paediatricians to consider as it can otherwise go undiagnosed for a long period of time. In this case report, we discuss a paediatric patient with GPA that presented with non-specific respiratory symptoms for several months and was then found to have pulmonary nodules on chest imaging once a broader differential diagnosis was considered. We will review the common presentation of GPA, the classification criteria and its management. This will ultimately assist any providers in identifying and managing GPA cases.