Chilaiditi sign and syndrome are uncommon conditions and often misdiagnosed. They are clinically significant, however, because they can result in a range of complications, including bowel volvulus, perforation and obstruction. When patients are symptomatic, treatment is usually conservative and surgery is rarely indicated unless there is a suspicion of ischaemia, or if conservative management does not resolve other signs and symptoms. This article describes Chilaiditi sign and syndrome, and presents four case studies to illustrate the relevant signs and symptoms.