Abstract
Recurrent hyperbilirubinaemia was described as a feature of familial Mediterranean fever in the 1950s and early 1960s. However, over the last 33 years only one case has been published. We present a 12-year-old Arab boy who developed recurrent hyperbilirubinaemia in the course of familial Mediterranean fever. His response to colchicine was excellent. Review of the literature reveals that hyperbilirubinaemia of familial Mediterranean fever has a distinct clinical picture characterized by concurrent peritonitis, minimal jaundice and short duration. Factors contributing to the paucity of reports in recent literature are discussed.