Pure arterial malformations are dilated, overlapping, and tortuous arteries forming a mass of arterial loops without a venous component. These malformations are rare, with limited cases reported in the literature. Affecting any of the intracranial arteries, they are usually found incidentally and often have a benign course.

A 28-year-old female with a history of infantile hemangiomas, polycystic ovary syndrome, and hyperprolactinemia presented with acne, mild hirsutism, and galactorrhea. Her neurological exam was normal. Magnetic Resonance Imaging (MRI) of the brain with and without contrast ruled out a prolactinoma but revealed a 3 cm vascular malformation at the margin of the left cavernous sinus extending posteriorly into the anterior left perimesencephalic cistern. Initial differentials included a pial arteriovenous malformation or less likely a dural arteriovenous fistula. A diagnostic cerebral angiogram revealed a pure arterial malformation without arteriovenous shunting. This was composed of dysplasia of the left posterior communicating artery and anterior choroidal arteries. Stenosis of the left internal carotid artery distally with collateral support into the left anterior cerebral artery and middle cerebral artery was also noted. Genetic testing for vascular malformation syndromes, including genes known to be associated with hereditary hemorrhagic telangiectasia, was negative. Repeat MRI/MRA done 8 months from the initial scan revealed no abnormal large arterial venous shunting or definite saccular aneurysm formation.

Although rare, pure arterial malformations should be considered in the differential diagnosis for patients presenting with a cranial vascular malformation. Management of these lesions is usually conservative given their lack of arteriovenous shunting and low risk of rupture, with only a few cases undergoing surgery or endovascular treatment.