Unusual variation of asplenia syndrome

The case of a 1-year-old cyanotic boy diagnosed with asplenia syndrome has been reported. By physical and laboratory examinations, levocardia, atrial inversion, primum ASD, single atrioventricular valve, single ventricle (left-hand morphology), rudimentary right ventricle (anterior, left-sided), pulmonary stenosis, left-sided vena cava, single vena cava superior were established and the case was diagnosed with asplenia syndrome. The patient has concordance between tracheo-bronchial situs and lung anatomy and inverted atrial and visceral situs, but without atrial isomerism that makes his case an unusual variation of asplenia syndrome.