Sickle cell disease (SCD) is a common genetic disorder which represents a major medical problem in certain parts of the world. It is characterized by chronic haemolytic anaemia and vaso-occlusive crises, which can lead to widespread vascular occlusion by sickled red blood cells leading to multiple organ infarctions. In this respect, SCD can be considered as a multisystem disease presenting, to various surgical disciplines, a wide variety of surgical ailments. As surgical management of these patients is associated with high morbidity and mortality, close collaboration between medical and surgical services is essential. Greater population mobility makes recognition of the surgical implications of this disease of paramount importance in surgical practice. This article reviews surgical problems with which SCD patients may present to general surgeons and discusses their management.