Familial lecithin:cholesterol acyltransferase deficiency is a rare autosomal recessive disorder associated with significant renal complications. A 16-year-old boy with the typical clinical and laboratory characteristics of this disease had a renal biopsy because of proteinuria and hematuria. The histologic findings were those of membranous glomerulonephritis with very few intracapillar foam cells. Ultrastructural study revealed numerous vacuolated deposits containing irregular profiles of electron-dense membrane-like material predominantly along with the subepithelial aspect of the glomerular basement membranes but also within the basement membranes, the subendothelial space, the mesangium, the capillary lumina, Bowman's capsule, and tubular basement membranes. The unusual aspects of this case are the relative paucity of intraglomerular foam cells and the predominantly subepithelial location of the lipid-like deposits.