OBJECTIVE To investigate pulmonary venous anomaly as a cause of pulmonary arteriovenous malformation (PAVM) misdiagnosis. METHODS We reviewed adult patients within a 7.5-year period with CT scans initially diagnosed with PAVM and subsequent conventional pulmonary angiograms. RESULTS Pulmonary arteriography showed no PAVM on arterial phase for 10 out of 99 patients, comprising the misdiagnosed group. Four misdiagnosed patients had pulmonary venous anomalies and six had nodular lesions on CT. CONCLUSIONS Pulmonary venous anomalies are vascular mimics of PAVMs that may lead to misdiagnosis. Optimal CT technique and careful imaging review are necessary to minimize inappropriate invasive angiography.