Granulomatosis with polyangiitis (GPA) manifests as necrotizing granulomatous inflammatory masses in the nasal cavity, paranasal sinuses, and lungs. However, a mass in the kidney is extremely rare. We herein report a case of GPA that presented as a solitary mass in the left kidney. The patient was a man in his 60s. A 2.5-cm solitary mass was incidentally discovered in the left kidney at another hospital and was followed-up. Eight months later, the mass had enlarged, and the patient visited our hospital for further examination and treatment. The mass was hypovascular, with unclear margins on contrast-enhanced computed tomography (CT). The signal of the mass was nonuniform and iso- to slightly hypo-intense on T2-weighted and diffusion-weighted magnetic resonance imaging (MRI). Enlarged para-aortic lymph nodes were also detected on the CT and MRI. Based on imaging, malignant tumors were suspected, and nephrectomy was performed. The pathological diagnosis was GPA. We performed a literature review of this rare renal manifestation and present a summary of reported imaging findings. If a hypovascular renal mass with an unclear margin can be found in those with GPA, unnecessary operations may be avoided by actively promoting renal biopsy.