Editor,—Apolipoprotein A-1 (Apo A-1) plays a central part in the metabolism of high density lipoproteins (HDL).1 Apo A-1 and apolipoprotein A-2 (Apo A-2) make up 80–90% of the protein content of HDL. The characteristics of this deficiency are low levels of HDL serum and Apo A-1, normal levels of triglycerides serum and high levels of LDL serum and total cholesterol.1-4 HDL concentration is inversely correlated with a risk of coronary heart disease (CHD).5 However, there is disagreement about the importance of normal Apo A-1 and HDL serum levels in preventing atherosclerosis.6 Signs of advanced atherosclerosis and early coronary heart disease were only found in some patients with Apo A-1 deficiency,3 5 and did not appear in other patients.4 6 Thus, in addition to low Apo A-1 levels, other cardiovascular risk factors must be present to cause premature atherosclerosis.5 7 Retinopathy, neuropathy, and corneal opacity are associated with this deficiency.5 8 9 Our report describes the corneal condition of a 37 year-old Sri Lankan woman with homozygous Apo A-1 deficiency.