Lecithin cholesterol acyltransferase (LCAT) deficiency, inherited or acquired, is characterized by markedly low plasma high density lipoprotein (HDL)-cholesterol levels and increased unesterified cholesterol. We report a case of an elderly patient with persistently very low HDL and proteinuria. Serum cholesteryl esters were markedly low, and kidney biopsy revealed diffuse global glomerular lipid deposition, classic for LCAT deficiency, whereas genetic testing for variants associated with LCAT deficiency was negative.