Internal jugular vein (IJV) phlebectasia is rare in occurrence and is frequently misdiagnosed and managed inappropriately. It commonly presents as a unilateral neck swelling which typically increases in size with valsalva maneuver. Although, the most common cause of a focal neck swelling, which increases in size with valsalva maneuver is laryngocele, the possibility of phlebectasia of IJV should always be borne in mind, especially in child. Owing to the rarity of this condition, a high index of suspicion is required to recognize the same and managed appropriately. We present a case of phlebectasia of the right IJV with intracranial extension and discuss its management. The case is being reported in view of its clinical rarity (the intracranial extension being extremely rare) and to highlight the available management strategies.