A case of infantile nephrotic syndrome (NS) with advanced membranoproliferative glomerulonephritis (MPGN), type I, and bilateral congenital glaucoma, is presented. The patient also had persistent thrombocytopenia and subclinical hypothyroidism. The parents were second-degree cousins and the affected infant had a sibling who was born with congenital glaucoma. His mother had an aunt and uncle on the maternal side who were born with congenital glaucoma. In addition, there was a history of infantile death in five family members of unknown causes (pedigree). To the best of our knowledge, the association of congenital glaucoma and infantile NS due to MPGN has not been reported previously.