While fenestration and duplication are relatively common in the arteries, they are extremely rare in the venous compartment: internal jugular vein fenestration has been reported occurring in 0.4% of unilateral neck dissections. Familiarity with these morphological anomalies is important for the radiologist and for the surgeon to prevent neurovascular injury, especially in neck surgery and interventional catheterization. We present the case of a patient harboring a fenestration of the left internal jugular vein, diagnosed by magnetic resonance angiography, and a systematic review of the literature. To our knowledge, from 1985 until 2016 only 36 patients (including the present) were diagnosed as having an internal jugular vein morphological anomaly. Out of 36 patients, only 11 (30,5%) were diagnosed using radiological imaging; the high rate of intra-operative diagnoses (22/36, 62,5%) is likely related to the limited use of diagnostic imaging or to misdiagnosis/misinterpretation of a relatively unknown and rare morphological anomaly. A contrast enhanced computed tomography or magnetic resonance angiography should be considered in case of vascular procedures in a patient with known internal jugular vein anomaly