Salivary gland choristoma of the middle ear in a child with situs inversus totalis

Salivary gland choristoma of the middle ear cavity is an extremely rare entity. It is thought to be a developmental abnormality and may be associated with abnormalities of adjacent structures. We report a case of salivary gland choristoma of the middle ear with prominent Körner's septum in a 7-year-old girl with situs inversus totalis. Situs inversus totalis is the mirror image reversal of the normal position of the internal organs and frequently associated with other congenital anomalies. This patient is the first reported case with situs inversus totalis, having middle ear salivary gland choristoma and prominent bilateral Körner's septae. The management and the differential diagnosis of this condition are discussed with the review of the literature.