Mucosal melanoma arising in the middle ear or eustachian tube is uncommon. We present a patient with hearing loss and otalgia found to have mucosal melanoma which occurred in the eustachian tube with extension into the middle ear cavity and external ear canal. Otologic clinics was consulted and biopsy of the mass located at the external canal was performed to ascertain the pathological diagnosis. The patient refused immunotherapy and surgery instead of undergoing radiotherapy and died from hepatic metastasis 8 months later. The mucosal melanoma originated from the eustachian tube with extension into the external ear canal is exceedingly rare, and the differential diagnosis should be considered for tumors in external ear canal.