Abstract
Three Saudi siblings, two girls and one boy, presented at the ages of 7, 6 and 2.5 years, respectively, and were diagnosed as having features of Indian childhood cirrhosis (ICC). The two girls presented at a late stage of the disease and the boy was diagnosed during routine examination of the family. The initial presenting complaint was abdominal distention and pruritus. All three had a rapid and fatal course. There was no evidence of increased copper ingestion by the families, supporting the suggestion of a hereditary metabolic role in the aetiology of ICC. As far as we are aware, this is the first report of ICC in Saudi Arabian children.