Salivary gland choristoma of the middle ear is a rare entity. It is believed to be a developmental abnormality that may be associated with anomalies of adjacent structures. We describe the case of a 6-year-old girl who had a salivary gland choristoma in the middle ear that was associated with an ossicular chain anomaly and a facial nerve anomaly. We discuss the clinical features and management of this condition, and we review the literature.