Object: The aim of this study was to describe a familial dyslipidemia revealed by a corneal arch.<span style="font-family:Verdana;"> </span><span style="font-family:Verdana;">Familial hypercholesterolemia is a rare and severe hereditary dyslipidemia with an exceptional homozygous form. He was a 43</span><span style="font-family:Verdana;">-</span><span style="font-family:Verdana;">year</span><span style="font-family:Verdana;">-</span><span style="font-family:Verdana;">old patient admitted with visual disturbance, xanthomatous papule and orange coloring at the level evolving for 5 years</span><span style="font-family:Verdana;">, </span><span style="font-family:Verdana;">no personal history of cardiovascular disease, having a family history of lipid disorders in siblings</span><span style="font-family:Verdana;">,</span><span style="font-family:Verdana;"> as a cardiovascular risk factor: age over 50 and the male gender</span><span style="font-family:Verdana;">,</span><span style="font-family:Verdana;"> normal cardio pulmonary examination, blood pressure at 120/80</span><span style="font-family:Verdana;"> </span><span style="font-family:Verdana;">mmhg, heart rate 78</span><span style="font-family:Verdana;"> </span><span style="font-family:Verdana;">bpm</span><span style="font-family:Verdana;">,</span><span style="font-family:Verdana;"> in whom the ophthalmological examination shows a yellowish spot on the cornea (</span><b><span style="font-family:Verdana;"><a href="#f1">Figure 1</a></span></b><span style="font-family:Verdana;">). We report the case of familial dyslipidemia revealed by a corneal spot in Guinea.</span><span style="font-family:;" "=""></span><span style="font-family:Verdana;">More recent studies increasingly suggest that it is much more widespread than previously thought: 1/200 to 1/400. Familial dyslipidemia is a rare and severe metabolic abnormality. It should be screened as early as possible like any other cardiovascular risk factor for atheroma to avoid the occurrence of a major cardiovascular event