We present our single-centre experience of two cases of lipochoristoma, a rare lesion causing unilateral hearing loss. Differential diagnoses include the more common vestibular schwannoma, haemangioma and meningioma. Diagnosis was confirmed with fat suppression sequences on MRI. Management of both patients was with serial imaging, under the guidance of neuro-otology multidisciplinary team, due to the slow and typically non-aggressive progression of this lesion.