Collagenofibrotic glomerulopathy (CG) is a rare renal disease with unknown etiology, defined by deposition of Type III collagen fibers in the subendothelial space and mesangium seen on supported by electron microscopy. There are merely 19 cases reported in the literature from the Indian subcontinent. Herein, we present a case report of CG from the Indian subcontinent and review its literature mainly focusing on histopathological findings.