Scimitar Syndrome is a congenital condition resulting in anomalous pulmonary venous drainage from the lung into the inferior vena cava. It is often associated with cardiac and pulmonary abnormalities, both which are determinants in the severity and timing of presentation. True incidence of this condition is unknown and likely to be significantly underestimated as many patients remain asymptomatic through adult life. The increase in availability and use of medical imaging suggests that more asymptomatic and subacute cases will be detected. The management in those with the delayed presentation of this condition is debated, however the mainstay of therapy remains medical.