The combination of a discordant atrioventricular connexion with a concordant ventriculo-arterial connexion (so-called "isolated ventricular inversion") is an extremely rare entity described, to the best of our knowledge, on only 15 occasions in patients with usual atrial arrangement. Initial diagnosis at cross-sectional echocardiography has only been reported three times, but the combination has been diagnosed in life on seven occasions, with successful Mustard or Senning operations having been performed in six of these patients. We report a neonate referred with an initial diagnosis of complete transposition, who was correctly diagnosed at cross-sectional echocardiography, and had closure of an associated ventricular septal defect with a Senning procedure in the neonatal period.