Congenital internal jugular phlebectasia corresponds to congenital dilatation of the vein without tortuosity. More than one hundred cases of phlebectasia involving the neck veins have been reported in the literature. The authors describe the clinical features, treatment and outcome of this anomaly.

A six-year-old child with no particular past medical history presented with a two-year history of progressively enlarging mass on the right side of the neck. Physical examination revealed a non-pulsatile mass that was increased in size by all manoeuvres increasing intrathoracic pressure. Neck computed tomography confirmed the diagnosis of internal jugular vein phlebectasia. In the absence of complications, simple surveillance was advised with a follow-up of 20 months with no complications.

Congenital internal jugular phlebectasia is a rare condition. Management must include imaging to confirm the diagnosis. Long-term surveillance is recommended and surgery is only required in the presence of complications.