Purpose <p>Myxomas are rare tumors arising from mesenchymal tissue and can manifest at different locations. Sporadic occurrence in the ear and temporal bone is extremely rare. We reviewed all the published literature about myxomas of the temporal bone region and presented clinical characteristics and management of a myxoma of the middle ear, spreading into the outer ear canal and mastoid in a 14-year-old boy.</p> Methods <p>We searched the PubMed database for all published case reports and case series describing patients with myxomas in the temporal bone region.</p> Results <p>Thirty-nine studies reporting myxomas of the temporal bone region were identified. The most common site is the skin of the auricle, but lesions may also originate in the external auditory canal, middle ear, temporalis muscle, or even the stapedial muscle. These tumors can erode the ossicular chain and adjacent bone and may extend intracranially. Radiologically, they can mimic other, usually benign, expansive lesions. Management is surgical. Some cases occur in association with Carney complex.</p> Conclusions <p>Myxomas of the ear and temporal bone are a rare pathology and can be mistaken for more common conditions, such as chronic ear inflammation. Although they are benign tumors, they can cause hearing loss, vertigo, headaches, and even cranial nerve palsy. Treatment is surgical. It is necessary to assess if they are associated with the Carney complex, as cardiac myxomas can be fatal.</p>