BackgroundChilaiditi’s sign is an incidental radiographic finding, associated with intestinal disposition located between liver and right diaphragm. It is considered as an acquired rather than a congenital condition and the prevalence ranges from 1.18% to 2.4% according to recent adult retrospective studies. The aspects of this rare entity with regards to a 7-month-old male initially misdiagnosed as diaphragmatic hernia is discussed.Case presentationA 4-month-old Caucasian male was misdiagnosed with a congenital diaphragmatic hernia owing to previous hospitalization with complaints of respiratory tract infection. On admission 3 months later, he was free of any signs and symptoms of intestinal obstruction or respiratory distress. Thorax computed tomography revealed Chilaiditi’s sign. A diagnostic laparoscopy was regarded necessary to evaluate the anatomical details. The most prominent finding was the lack of muscle fibers and almost transparent appearance of the medial aspect of the partially eventrated right hemidiaphragm. Owing to delicate anatomical presentation, diaphragmatic plication was considered hazardous. The patient is doing well and under follow-up.ConclusionsIt is obvious that Chilaiditi’s sign is not always a completely incidental finding of no consequence, and may indicate an underlying congenital diaphragmatic pathology, clearly defined by laparoscopic evaluation in this case.