Mild liver dysfunction occurs in patients with coeliac disease frequently, but severe liver diseases in combination with coeliac disease are rare. A 19-year-old woman was referred for evaluation for emergency liver transplantation with progressive jaundice and prolongation of prothrombin time. She had a history of non-bloody diarrhoea for 3 months and weight loss of 12 kg. At admission, abdominal ultrasound revealed liver enlargement with increased echogenicity and marked thickening of the circular folds of the small intestine, suggestive of coeliac disease. The diagnosis was confirmed by endoscopic biopsy from the second part of the duodenum. On a gluten-free diet the patient recovered within 1 week and remained in excellent health up to the present date. Liver tests normalized within 5 months and a repeat abdominal ultrasound after 20 months revealed a normal liver and small intestine. This case report suggests that coeliac disease should also be considered in patients with unexplained severe acute liver disease and that abdominal ultrasound may be a helpful step to obtain to the correct diagnosis.