✓ The authors report a case of a 45-year-old woman with pulsatile tinnitus who was found to have an unusual spontaneous fistula between the petrous internal carotid artery and internal jugular vein. The fistula resolved spontaneously, possibly related to daily manual compression of the ipsilateral common carotid artery. The patient also had a contralateral carotid artery dissection, multiple intracranial arachnoid cysts, and hemifacial atrophy. Her medical history was significant for easy bruisibility, abnormal scarring, and mitral valve prolapse. This association appears unique and may represent a previously undescribed generalized connective tissue disorder.