A case report of a six-year-old child is presented, who had had a unilateral sensorineural hearing loss for several years. Because of impairment in the ABR as well as in the caloric testing a MRI and CT scan were performed. A 17 mm tumour in the cerebellopontine angle (CPA) was detected, which after suboccipital surgery proved to be a rhabdomyoma. This tumour has not been described before in the CPA. Unilateral sensorineural hearing loss should, at all ages, be an indication for further (radiodiagnostic) investigations.
