We report here a case of severe membranoproliferative glomerulonephritis with a singular high titer of anticardiolipin antibody (aCL). A 19-year-old Japanese female was admitted to Tsukuba Gakuen Hospital after complaining of general edema for 5 months. She had no past history of thrombosis, thrombocytopenia, or spontaneous abortion. Laboratory findings revealed that she had nephrotic syndrome and moderate renal dysfunction. Immunological test showed a high titer of aCL with a high-normal limit of antinuclear antibody, negativity for anti-beta(2) glycoprotein I antibody and negativity for anti-DNA antibody. In the renal biopsy tissue, most glomeruli showed global sclerosis and the remaining glomeruli revealed membranoproliferative change with crescent formation. Steroid therapy with warfarin and dipyridamole was effective and her renal function improved gradually. This case lacked the typical symptoms of primary antiphospholipid syndrome and did not satisfy the criteria of SLE. In spite of these findings, the singular high titer of aCL with membranoproliferative glomerulonephritis characterized this case.