In this report, we describe a 41-year-old woman with symptoms concerning for acute carpal tunnel syndrome. Operative exploration resulted in resection of an aneurysmal, thrombosed, persistent median artery seen on preoperative imaging and full recovery by the patient. A persistent median artery is an uncommon congenital hand condition resulting from the failure of the median artery to regress, which can then travel through the carpal tunnel and be associated with a bifid median nerve, irritation of the nerve, and need for surgical exploration if it thromboses, which is rare. This should be considered in patients with acute median nerve compression, without other etiologies.