Introduction Klippel-Feil Syndrome is a complex condition, characterized by fusion of cervical vertebrae 2 and 3; however, it has been shown in several studies that fusion of the vertebrae also affects different cervical vertebrae. It is caused by a failure of the normal division or segmentation of the cervical vertebrae in early fetal development. This anatomical variant can lead to chronic headaches, limitation of neck movement and pain in the neck muscles. In addition, it may manifest neurological symptomatology (1)(2). The aim of this literature review is to consider this malformation as one of the main differential diagnoses in patients who present neurological symptoms or a history of trauma; since, as has been shown, the presence of this malformation can be confused with the presence of a cervical fracture; therefore, patients who present with clinical or imaging alterations need to be carefully evaluated to confirm or rule out the presence of this pathology. Conclusions Klippel-Feil, being a very rare pathology, is often forgotten, so the doctor must remember the most important clinical-epidemiological characteristics, since this pathology can be considered as a differential diagnosis in patients with a pathological condition located in the cervical region; in addition, this pathology should be considered when the patient has systemic pathologies, especially of the renal type.