Variations in the course of the internal carotid arteries (ICA) are reported in the literature as coiling, looping, kinking or tortuosities of the vessels. Nevertheless, the definitions between these variants are confusing. Also, the clinical relevance of morphological anomalies of extracranial ICA is a matter of debate because of up to date the natural history of kinking, coiling and tortuosities of this artery is not well known. However, some authors consider that these conditions are burdened with disabling, even fatal neurological complications. Also, variations of the ICA cervical course may lead to direct contact of the artery with the pharyngeal wall, being of great clinical relevance due to the large number of routine procedures performed in this region. In the present study, we describe two cases of ICA tortuosities and review the current literature regarding the causes, symptoms and clinical significances of the variations of the cervical ICA course. Tortuosity of the cervical ICA is not a rare condition and they can easily be mistaken clinically for an aneurysm, a tumor or an abscess and subsequently injured during an attempted biopsy or excision. Thus, regardless the controversy of its causes (congenital or acquired) it should be included in the differential diagnosis of cervical soft tissue widening. Also, they should be taken into consideration on the diagnostic procedures for ischemic transitory attacks and/or stroke.