Introduction: Neck veins are increasingly used in autologous tissue transfers, diagnostic procedures, and intravenous therapies. Thus, knowledge of variation in their anatomy is of huge clinical importance in modern medicine. There have been several case reports of variation in the external jugular vein (EJV) anatomy, but fenestration remains one of the rarest anomalies reported. Case Report: During dissection of an embalmed 80-year-old male cadaver, unilateral (left-sided) fenestration of the EJV was noted. Shortly after the union of the retromandibular and posterior auricular veins, the EJV bifurcated, forming medial and lateral divisions. These descended superficially to sternocleidomastoid muscle before reuniting inferior to its posterior border in the posterior triangle of the neck and draining into the subclavian vein. No structures passed through the fenestration. A review of existing literature found five reports of unilateral EJV fenestration. Of note, ours is the only case where the anomaly has occurred in a male. Conclusion: Fenestration of the EJV is a rare anatomical anomaly. This case report contributes to the limited body of evidence regarding the true incidence of this variant. Further research will be beneficial in building a more comprehensive