| Summary: | Congenital malformations of the external iliac arteries are rare and have been reported few times in the literature. Some of these cases have been associated with other organ anomalies, and to our knowledge only one reported case was associated with a congenital heart defect. Here we present a case of a patient with a congenital heart defect, Tetralogy of Fallot, and malformation of an external iliac artery. A 38-year-old woman with a past medical history of Tetralogy of Fallot, severe levoscoliosis, unspecified autonomic dysfunction and no smoking history presented with complaints of worsening right lower extremity effort induced pain for the past year. On exam, her right lower leg circumference was noticeably smaller than the left, measuring 29 cm and 31 cm, respectively. Pulses were palpable in the left leg, but no femoral pulses were palpable in the right. Computed tomographic angiography (CTA) showed absence of the right external iliac artery (EIA) with reconstitution via iliolumbar collaterals into the right common femoral artery (CFA) which was of normal caliber. At surgery it was revealed that the right EIA was atretic and remained as a cord-like-structure with no patency.
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