Academy of Medical Sciences of I.R. Iran Archives of Iranian Medicine 1029-2977 23 6 2020 06 01 Collagenofibrotic Glomerulopathy: Report a Rare Case 409 411 10.34172/aim.2020.35 EN Rakesh Agarwal Rashmi Baid Jotideb Mukhopadhyay Journal Article 10.34172/aim.2020.35 2018 07 29 2019 02 11 Collagenofibrotic glomerulopathy is a rare clinical entity with fewer than 40 cases reported worldwide. We describe a case of adult onset nephrotic syndrome with partial villous atrophy of the intestine who was diagnosed with this rare entity. Collagenofibrotic glomerulopathy is characterized by subendothelial and mesangial collagen type III deposition and increased procollagen III peptide levels. Extra renal involvement has been described in the form of hypertension, anemia and microangiopathic hemolytic anemia but villous atrophy has not been associated with this condition so far, possibly reflecting the paucity of literature. We describe this case and review the condition in this report.