We aimed to investigate the incidence, etiology, treatment, and clinical course of atypical iliac vein compressions (AIVCs) among patients with May-Thurner syndrome (MTS).A total of 173 patients who presented with MTS were retrospectively analyzed at a single center. Computed tomographic venography (CTV) was used to diagnose MTS. An AIVC was defined as the compression of the left common iliac vein (LCIV) by structures other than the right common iliac artery (RCIA) or the compression of other venous structures in the pelvic cavity instead of the LCIV. The patients with AIVC were categorized into the LCIV compression group (category A) and non-LCIV compression group (category B).Ten patients with AIVC were identified (5.8%; male/female, 5/5), five in category A and five in category B. The median age of patients was 76 years (range, 51-94 years), and the median follow-up duration was 388 days (range, 12-4694 days). In category A, the LCIVs were compressed by the left common iliac artery (LCIA) (n=2), uterine leiomyoma (n=1), LCIA aneurysm (n=1), and RCIA aneurysm (n=1). In category B, the right common iliac veins were compressed by the RCIA (n=4) and L5 osteophyte (n=1). Endovascular treatment, including balloon angioplasty and stent placement, was performed in six patients, three from each group. Three patients underwent conservative treatment due to their advanced age and comorbidities. Endovascular aneurysm repair was performed in one patient with RCIA aneurysm. Follow-up images were available for six patients, and all of them had patent venous flow.The AIVC had an incidence of 5.8% (10/173) among symptomatic MTS patients and wide spectrum of etiologies. Pathogenesis-tailored endovascular treatments are safe and effective.