This case report investigates the genetic basis of collagenofibrotic glomerulopathy (CG), a type of collagen type III glomerulopathy. It is a rare kidney disease characterized by collagen III deposition. A 47-year-old man with CG, born to consanguineous parents, underwent whole-exome sequencing. A homozygous truncating variant in STAB2 and a heterozygous variant in STAB1 were identified. Neither of the proteins encoded by STAB2 and STAB1 was expressed in the kidney glomeruli, suggesting a systemic mechanism for CG development.