Jugular phlebectasia is an entity that is being increasingly recognised in recent years. The term phlebectasia indicates dilatation of the vein without tortuosity. It has been described in almost all cervical veins. Internal jugular phlebectasia is seen more often on the right side. This paper reports two new cases, reviews all cases of internal jugular phlebectasia in children published in English literature upto 1996 and recommends diagnostic methods and treatment policy. This article also discusses a theory of pathogenesis for this condition based on the regional anatomical features, principles of vascular physics and pathologic findings.