A 60-year-old woman was referred to our hospital for the evaluation of a 2.2 cm-sized focal consolidation with an air bronchogram sign incidentally detected by non-enhanced chest computed tomography (CT) (
Fig. 1A). The patient’s medical history, physical findings, and laboratory test results were unremarkable. Contrast-enhanced CT showed a well-enhanced vascular structure (
Fig. 1B). Pulmonary arteriography revealed a cut-off sign in the left pulmonary artery (
Fig. 1C). Aortography revealed a dilated vascular structure from the left inferior phrenic artery (IPA), and selective angiography confirmed a fistula between the left IPA and left pulmonary vessels (
Fig. 1D). Because there were no apparent symptoms, the patient was discharged and scheduled for outpatient follow-up. However, she did not visit for 49 months until she was referred for persistent lesions. Contrast-enhanced CT revealed engorgement of the left IPA with a fistula (
Fig. 1E), and selective angiography revealed dilatation of the feeding artery with more prominent collateral vessels (
Fig. 1F). Based on these findings, transcatheter embolization of the left IPA was successfully performed. Systemic artery-to-pulmonary vessel fistula (SAPVF) is a rare vascular anomaly for which the disease course is unclear. Although most patients are asymptomatic [
1], they can develop life-threatening conditions, such as hemoptysis and pulmonary hypertension [
2]. Our case highlights that SAPVF can manifest as a growing chronic consolidative lesion with an air bronchogram, possibly due to dilatation of the pulmonary vessels.