Hypoglassia or aglossia is an uncommon anomaly, either of which may occur as an isolated finding or in association with other deformations, especially limb anomalies. Their genetic background is uncertain, and drug induced teratogen has not been clearly identified. We experienced a case of congenital aglossia with situs inversus in a female infant aged twelve days. Her initial complaints at admission were feeding difficulty and weight loss. In a review of literature, the association with situs inversus is very rare and only three cases have been reported until now.