The Zieve syndrome is characterized by hemolytic anemia in conjunction with secondary hyperlipidemia in patients suffering from alcohol-related toxic liver damage. So far no retinal changes have been reported due to the Zieve syndrome.A 44-year-old diabetic attended a routine control by an ophthalmologist. It was known that he had steatohepatitis due to an abuse of ethanol for years and 1 month previously he had suffered from nausea and vomiting. The visual acuity was found to be good on both sides (1.0/0.8). In the fundus white-colored retinal vessels and peripapillary cotton wool exsudates with isolated hemorrhages were found. An extremely lipemic serum and a severe anemia were present. The patient was referred to the internal department for further investigation and because of the anemia the patient received two blood transfusions. The elevated cholesterol and triglyceride levels in the serum could be substantially reduced by treatment with a cholesterin synthetase inhibitor and a fibrate. The follow-up examination 3 weeks later revealed a regular blood flow and a regression of cotton wool exsudates and 1 month later no pathological retinal findings were detected.The retinal changes were caused by the combination of severe anemia and hyperlipidemia and these findings played a leading role for deciding further diagnostic procedures. After treatment of the metabolic disorder the retinal changes were completely reversible without functional restrictions.