Pseudoexfoliation (PE) syndrome in addition to inherited systemic amyloidosis with lattice corneal dystrophy is reported in 1 clinical case, and in 5 cases out of 6 autopsies. Histochemical evidence of amyloid deposits were found in the PE material accumulated adjacent to the ciliary body, the lens capsule, and the iris surface. These new observations suggest that PE accumulation has been only a single phenomenon among others during amyloidogenesis of this familial type.