A case of discordant atrioventricular (AV) connection and concordant ventriculoarterial (VA) connection (with aortomitral fibrous continuity and tricuspid pulmonary discontinuity) in situs inversus (I, D, I) is reported because of its rarity. This segmental combination with abnormally related great arteries in the absence of anomalies of venous connections and/or splenic anomalies is extremely rare.