The occipital artery (OA) typically originates from the external carotid artery (ECA). Variations of the ECA has been well described in the current literature, while the OA is a relatively stable vessel, and its variations are uncommon. In the current case report, an aberrant OA has been found coexisting with a linguofacial trunk (LFT) on the right hemineck of a 51-year-old male patient. The OA was identified originating from the cervical internal carotid artery (ICA) at the level of the second cervical vertebra (C2). On the ECA, the lingual and facial arteries were emanating in common, as LFT. The left hemineck of the patient was free of variations. The current coexistence of arterial variants has been reported only once previously; therefore, the current case corresponds to the second case in the English literature. The aberrant OA origin from the ICA has been estimated with a pooled prevalence of 0.37%, while the origin at the C2 level and from the anterior surface of the ICA corresponds to a very rare variation. Additionally, the LFT is one of the most common trunk that can be found on the ECA. Interventional radiologists and surgeons must be aware of common and uncommon variation to avoid iatrogenic lesion.