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The value of ophthalmological examination is often overlooked when we deal with disorders of hepatobiliary system. There is a wide spectrum of genetic, metabolic and other acquired disorders in infants and children that have ocular changes along with hepatic involvement. If the disease course is insidious then these findings may be noted in adolescence or early adulthood in several such conditions. Some ocular findings are very suggestive of the underlying etiology giving the clinician an opportunity for early diagnosis and intervention. Where the condition is untreatable, decision regarding palliative measures, prognostic counselling and genetic counselling for future pregnancies can be offered earliest to the caregivers. The article attempts to discuss the ophthalmological findings of several such disorders manifesting in infancy and childhood. Pediatricians, ophthalmologists and even physicians dealing with adult medicine should be aware of these findings.