In this small case series, Casazza et al describe their management of seven cases of complex cholesteatoma presenting during a 16-year period. Patients were included if imaging confirmed restricted diffusion and an endophytic, medially-destructive disease involving the otic capsule, petrous apex or skull base. Mean age was 41.3 years. Patients presented with hearing loss and other symptoms of complex chronic ear disease (e.g., facial nerve weakness or intracranial involvement). Five patients had undergone previous otological surgery, two for congenital cholesteatoma. Imaging demonstrated tegmen dehiscence (n=1), lateral carotid dehiscence (n=2), petrous apex involvement (n=2), lateral, superior or posterior semi-circular canal dehiscence (n=4), erosion into the cochlea (n=1), and facial nerve dehiscence (n=1). Surgical approaches were dictated by the disease and included infratemporal fossa with facial nerve rerouting (n=2), trans-labyrinthine (n=1), trans-cochlear (n=2), middle cranial fossa with trans-mastoid (n=1), and trans-mastoid alone (n=1) approaches. Five patients underwent blind sac closure. Postoperative complications included CSF leak (n=1) and deterioration of pre-existing facial weakness (n=1). Mean follow-up was 21.1 months, with serial diffusion-weighted imaging to assess disease status. Two patients underwent second-look procedures. Residual disease medial to the facial nerve or geniculate ganglion was identified in two patients, one on imaging, one during second-look surgery. This series nicely highlights the surgical and follow-up challenges posed by complex cholesteatoma that invades medially.