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Background: Intercostal artery aneurysms (ICA) are rare vascular disease. A rupture of ICA is a possible mechanism of intramural aortic hematoma (IH). We report a case with IH and ICAs without clear etiology. Case presentation: A 64-year-old man was admitted to our emergency room with a sudden onset of acute diffused abdominal and chest pain, radiating to the back. Without previous traumatic insult, a computed tomographic angiography scan (CTA) revealed an IH beginning inferior to the left subclavian artery extending to the level of the celiac trunk. Follow-up CTA demonstrated a stable maximal IH thickness diameter of 11 mm, maximal aortic diameter of 40 mm, a new left hemorrhagic pleural effusion, and a focal contrast enhancement at T9 level. Due to these findings, thoracic endovascular aortic repair (TEVAR) was performed. During follow up, T9 focal enhancement continues to grow and an additional one developed. Selective angiography was performed demonstrating a connection to the costal artery and the aortic lumen, confirming ICA. Successful embolization with micro coils was performed. During follow up, additional 2 ICAs developed and treated with embolization. CTA three months later showed a complete resolution of the IH and obliteration of all treated ICAs. Infectious, inflammatory and connective tissue disease investigations were undertaken without a clear etiology. Conclusions: This is a case of IH and ICAs, in the absence of a clear etiology which were successfully treated by endovascular procedures TEVAR and coil embolization. It is not clear whether the hematoma was the source of the ICA or the other way round. Lack of ICAs in the initial CTA might be due to the pressure exerted by the hematoma or that they were too small to be detected but continued to grow on follow up. Rupture of these micro-aneurysms is a possible mechanism of intramural aortic hematoma. [ABSTRACT FROM AUTHOR]