Double outlet right ventricle, pulmonary stenosis and congenital mitral stenosis

A 4-year-old girl child was diagnosed with double outlet right ventricle (DORV), severe pulmonary stenosis, and supramitral ring. This case is presented to bring to light this rare association. Through this report, we aim to stress importance of assessing mitral apparatus on echocardiography during evaluation for situations like DORV and Tetralogy of Fallot (TOF). The physiological differences in such situations as opposed to their isolated counterparts and special postoperative outcomes are also discussed.