Scimitar syndrome with bilateral abnormal venous drainage and horseshoe lung is extremely rare. These rare complex anomalies were diagnosed in a 5-year-old boy by 64-slice multidetector CT (MDCT). This technique provides high-quality visualization of vascular, bronchial and parenchymal structures in a single session, such that no further invasive techniques are required. One obvious disadvantage of MDCT is the radiation exposure, especially in paediatric patients. The use of a single phase of contrast material administration reduces radiation exposure. The workstation platforms of MDCT systems allow multiplanar 2-D and 3-D postprocessing. As a result, various complex pathologies, such as that discussed here, can be diagnosed following a single imaging session with a certain precision.