ObjectiveTo present a case of giant cholesteatoma and review of the contemporary literature.PatientsA 61-year-old male presented with foul-smelling discharge from his right ear in the setting of a previous canal wall down mastoidectomy. Otomicroscopy demonstrated a mastoid cavity filled with infected keratinous debris. Neuro-imaging revealed soft tissue inflammatory changes in the mastoid cavity, with involvement of the temporomandibular joint (TMJ) and parotid gland and tegmen erosion. Biopsies showed evidence of desquamated epithelium consistent with cholesteatoma, with no malignant cells identified. Audiogram demonstrated a mean hearing loss of 65 dB on the right ear and an air-bone gap of 45 dB. Review of literature was also performed on giant cholesteatoma.InterventionsHe underwent a modified Fisch Type B infratemporal fossa approach to completely remove the cholesteatoma and a vascularized free flap was utilized to fill the surgical defect.Main Outcome MeasuresComplete extirpation of choles-teatoma and resolution of otorrhoea.ResultsRepeat imaging showed complete removal of cholesteatoma and clinically there was resolution of the otorrhea. Review of the literature has shown a lack of consensus around the definition of giant cholesteatoma. Most cases in the literature demonstrated extensive involvement of anatomical spaces beyond the confines of the temporal bone to qualify for the diagnosis of giant cholesteatoma.ConclusionsGiant cholesteatoma should be suspected in cases of recurrent ear discharge following canal wall down mastoidectomy. The case we report add to the literature to benefit future patients in preoperative counseling and better inform management.