Klippel-Feil syndrome (KFS) is a rare congenital cervical synostoses. Originally, it was believed that a correct determinant for the diagnosis of this disease to occur the patient must possess a 1) short neck, 2) low occipital hairline, 3) limited cervical mobility. More recently, most authors consider that any patient who has failure of segmentation of two or three cervical vertebrae may be categorized as KFS. This syndrome is usually found in a young age and is probably associated with some congenital anomaly. Our patient combined KFS with dextrocardia and forked rib is a extremely rare case, so offering the purpose of further study.