The presence of mitral regurgitation (MR) in patients with heart failure represents an independent predictor of mortality. Until now, the standard therapy for severe MR has been cardiac surgery in order to perform mitral valve replacement or mitral valve repair. With the introduction of the MitraClip® system (Abbott Vascular - Structural Heart, Menlo Park, CA, USA) in 2008, there is now an alternative percutaneous treatment option available for high-risk patients. We report on an 84-year-old male patient who was admitted to the emergency room with increasing shortness of breath due to severe functional MR, at stage NYHA III. In the following days the patient developed cardiogenic shock due to failure of the diuretic medication. The case first described here demonstrates an alternative transseptal route of access via a direct atrial puncture of the RA in a patient with absent inferior vena cava. This approach was successfully used to perform a MitraClip® procedure in this patient with functional MR and cardiogenic shock. It was possible to treat mitral regurgitation and the consecutive cardiogenic shock by implanting two MitraClips®.