Left side atrial anomalous pulmonary venous connection (PAPVC) is a rare entity. We report one case surgical treated off-pump with minimally invasive approach. We present a case of left side PAPVC with a non-significant atrial septal defect (ASD) in an asymptomatic 40-year old patient. Angiographic computed tomography (CT) scan has confirmed the diagnosis of vertical vein between left superior pulmonary vein and left brachiocephalic vein, responsible of a significant shunt and right heart dilation. Surgical repair consisted of implantation of the vertical vein into the left appendage through a left mini-thoracotomy and video-assistance. Outcome was event free and patient was discharged on day 4. Conclusion: Off-pump minimally invasive surgical repair of left side PAPVC is safe, effective and easily reproducible. Possible associated ASD may be treated with hybrid approach and percutaneous closure if indicated.