INTRODUCTION: Isolated iliac artery dissection (IAD) is a rare form of arterial dissection. The most commonly known causes of non-traumatic isolated iliac dissection are connective tissue diseases. Relapsing polychondritis (RP) is a rare inflammatory and multisystemic disease, typically affecting cartilage and connective tissue. Cardiovascular complications occur in approximately 25% of patients with RP. CASE DESCRIPTION: The first case of spontaneous isolated bilateral iliac artery dissection in a patient with RP is reported. A 48-year-old woman presented with a two year history of severe right leg intermittent claudication (Rutherford category 3). The complaints were initially attributed to joint pathology associated with RP. However, clinical examination and computed tomography angiography led to a diagnosis of bilateral IAD. A dissection in the left common iliac artery (CIA) began at its origin and ended at the origin of the internal iliac artery, with the true and false lumen both comprising half of the entire lumen. The right CIA was completely occluded from its origin to the origin of the right internal iliac artery. The patient was treated endovascularly by bilateral CIA stent placement with covered stent grafts (Bentley BeGraft), 8 × 57 mm on the right side and 8 × 37 mm on the left. The patient recovered pedal pulses, the stent remained patent, and the patient was asymptomatic at the one month follow-up. DISCUSSION: Early identification of intermittent claudication is necessary to prevent the progression of complications in patients with RP. Vascular surgeons should be aware of IAD as the potential first presentation of underlying systemic disease. Internists should also keep vascular complications in mind in patients with systemic diseases like RP suffering from unexplained complaints in the lower limbs, undertake a basic vascular examination, and make a vascular referral where appropriate.