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In this paper, the case of a 34-year-old male with sickle cell disease, recurrent episodes of ‘girdle syndrome’, and development of chronic ischaemic colitis is reported. At his last admission to the hospital, he presented with ileus attributed to severe intestinal ischaemia. During his hospitalisation, despite optimal supportive treatment, he developed acute liver failure, possibly as a result of acute intrahepatic cholestasis, a rare but fatal complication of sickle cell disease, and died from sepsis and multiorgan failure.

‘Girdle Syndrome’ Progressing to Ischaemic Colitis and Acute Intrahepatic Cholestasis in a Patient with Sickle Cell Disease: A Case Report