Granulomatosis with polyangiitis vasculitis (GPA), or formerly known as Wegener’s Granulomatosis (WG) and sarcoidosis are two distinct granulomatous diseases characterized by multisystem involvement. The sequential development of these two diseases is very rare. We report a patient who initially exhibited the symptoms of sarcoidosis and then limited GPA, predominantly affecting the nose and paranasal sinus, which was histologically proven. Imaging, pathological, and laboratory findings were described. After treatment, controlled clinical symptoms and changes in laboratory findings and imaging were demonstrated.