Tetra-amelia is a rare congenital disorder characterized by the absence of limbs. We describe the anesthetic management of a 29-year-old woman with tetra-amelia who underwent general anesthesia for tympanomastoidectomy with meatoplasty for an extensive right ear cholesteatoma. Anesthetic challenges related to tetra-amelia include difficult intravenous access, lack of sites for blood pressure monitoring, and possible difficult airway management due to craniofacial anomalies. Our case report focuses on the complex establishment of iliac artery access for invasive blood pressure monitoring by initially cannulating the carotid artery.