Regardless of the underlying pathology, elevated intracranial pressure is the endpoint of any impairment in either cerebrospinal fluid (CSF) absorption (including arachnoid villi) or intracranial venous drainage. In all age groups, the predominant final common pathway for CSF drainage is the dural venous sinus system. Intracranial venous hypertension (ICVH) is an important vascular cause of intracranial hypertension (and its subsequent sequelae), which has often been ignored due to excessive attention to the arterial system and, specifically, arteriovenous shunts. Various anatomical and pathological entities have been described to cause ICVH. For the second time, we present a unique case of severe focal stenosis in the distal sigmoid sinus associated with concurrent hypoplasia of the contralateral transverse sinus causing a significant pressure gradient and intracranial hypertension, which was treated with endovascular stent placement and angioplasty.