Background: Levamisole is a steroid sparing agent used in the treatment of steroid dependent and frequently relapsing nephrotic syndrome. It is used in combination with low dose prednisolone or as monotherapy. Though generally being a safe medication levamisole is known to cause rare systemic side effects like antineutrophil cytoplasmic antibody (ANCA) positive cutaneous vasculitis. Methods: The clinical, biochemical and histopathological data of 3 cases of levamisole induced ANCA positive glomerulonephritis at our institution were reviewed. Results: We report 3 patients with nephrotic syndrome treated with levamisole who developed ANCA positive glomerulonephritis. All of them had normal C3 and C4. None had severe forms with associated crescents or global glomerular involvement. Conclusions: Levamisole induced glomerulonephritis is rare and a high index of clinical suspicion, early evaluation and aggressive management are important in the management of these patients.