We describe a neonate with a rare congenital anomaly of the aorta. The anomaly included a hypoplastic aortic arch that was cervical and right sided. This complex combination was treated by a Norwood type procedure reconstructing a right-sided arch and, in a later stage, a Rastelli procedure. These 2 procedures achieved a 2 ventricular repair. The diagnostic and surgical challenges of this rare anomaly are described in this case report.