A case of unilateral complete aplasia of the petrous apex and inner ear associated with H-type tracheoesophageal fistula is reported. polytomography, computerized axial tomography, and plain film radiography confirmed the presence of this rare variant of inner ear aplasia. This patient, who has normal facial nerve function, is, to our knowledge, the first reported case of a nonthalidomide, Michel-type aplasia since the original report of Michel more than a century ago. Unilateral complete inner ear aplasia associated with tracheoesophageal fistula has not been previously reported.