Despite the cardiac surgery improvements allowing the correction of the majority of congenital heart diseases with ventricle-great vessels discontinuity, some abnormalities increase the risk of bi-ventricular reparation. We herein report the case of a patient presenting a rare form of double outlet right ventricle with a ventricular loop, with moderate right ventricle hypoplasia. L-malposition of great vessels and pulmonary artery stenosis, and for whom we opted for a palliative surgical treatment including a systemic-pulmonary anastomosis followed by a upper right bi-directional cavo-pulmonary derivation. The last surgery was followed by recurrent right pleural effusions disappearing after the embolization of the systemic-pulmonary anastomosis by catheterism as it probably obstructed the draining of the cavo-pulmonary anastomosis. The relevance of this clinical case reported is, firstly the description of this ventricle loop resulted from a marked ventricular malposition which is a rare heart disease, and secondly the discussion about the surgical treatment, especially about the choice between palliative and curative surgery. Only comparative studies on long term morbidity and mortality between the bi-ventricular reparation and mono-ventricular palliation will allow the selection of the most appropriate surgical treatment.