A 13-year-old boy presenting with digital and lip cyanosis, easy fatigability, and weight gain was diagnosed to have an intrapulmonary arterio-venous fistula. During his routine follow-up examinations, there was fullness on right upper quadrant of his abdomen. Abdominal ultrasonography (USG) showed a mass in portal hilus. We planned abdominal computed (CT) to gain tomography detailed information about the mass. Surprisingly abdominal CT and the color-Doppler sonography showed that the structure mimicking a mass was patent ductus venosus (PDV). We report PDV with intrapulmonary arterio-venous fistula as a unique and rare clinical entity. In addition, we underline the misdiagnosis of portal mass instead of patent ductus venosus. If there is a portal mass in USG or CT, color-Doppler sonography or, if needed, other diagnostic methods should be used to exclude PDV.