Granulomatosis with polyangiitis is characterized by necrotizing granulomatous inflammation, vasculitis with vascular lesions of small and medium caliber and focal necrotizing glomerulonephritis. A frequent and one of the most formidable complications is kidney damage, which in a large number of cases leads to a complete loss of organ function and a switch to renal replacement therapy. Given the rare occurrence of this disease in the clinical work of practitioners, and their low awareness of this pathology, problems often arise with the diagnosis and treatment of patients with HPA. Due to the diversity and non-specific nature of the manifestations of the disease, a delay in diagnosis may occur. The presented case illustrates the manifestations of granulomatosis with polyangiitis in the form of severe damage to the upper respiratory tract and kidneys, the diagnosis of which was difficult due to the rarity of the disease and the multiple organ pathology. This article presents a clinical case of severe progression of rapidly progressive glomerulonephritis in a patient with ANCA-associated vasculitis, a brief review of the literature is given. Despite adequate therapy, the disease progressed mainly due to deterioration of renal function, which subsequently led to a complete loss of kidney function and the transition to treatment with chronic hemodialysis.