Aim: We present here a case of facial schwannoma co-existent with an external auditory canal osteoma which presented with a history of unilateral facial asymmetry with conductive hearing loss. Case Report: A 50 year old female presented with progressive unilateral facial asymmetry of the left side since 5 years, with gradually progressive hearing loss in the left ear since 2 months. Examination revealed left-sided unilateral lower motor neuron facial palsy and a whitish bony hard mass in the left external auditory canal. CECT and CEMR scan of temporal bone showed a neoplasm arising from the vertical segment of left facial nerve. Modified radical mastoidectomy was done to trace the facial nerve lesion along the vertical segment of the facial nerve in the temporal bone and the exploration was extended along its extratemporal segment. The neoplasm was excised and histopathology examination revealed facial nerve schwannoma. The bony mass in the external auditory canal was excised and histopathological examination confirmed it to be an osteoma. Conclusion: The gradually progressive unilateral facial asymmetry was due to the facial nerve schwannoma, while the unilateral conductive hearing loss was explained by the presence of the osteoma in the external auditory canal. The patient was cleared of the disease and physiotherapy was started. The asymmetry of the face improved from House-Brackmann grade 5 to 4 within a month. The hearing threshold improved to within the normal hearing range. The patient has been followed up for 6 months and there is no recurrence of symptoms.