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To review an institutional experience with the surgical and clinical management of acquired middle ear cholesteatoma in patients with ectrodactyly, ectodermal dysplasia, cleft lip/palate (EEC) syndrome.Retrospective chart review.Tertiary referral center.Eight patients with medical history significant for EEC syndrome who underwent surgery for acquired middle ear cholesteatoma between 1996 and 2016.Appropriate surgical interventions at the time of admission.History of ventilation tube insertion, status of the contralateral ear, surgical technique, cholesteatoma recidivism, presence of postoperative external auditory canal stenosis, pre and postoperative audiograms.Cholesteatoma was diagnosed in all patients, 3 (37.5%) unilateral and 5 (62.5%) bilateral, totalizing 13 ears. Six ears (46.2%) underwent a canal wall up mastoidectomy but required conversion to a canal wall down technique in a second procedure due to recurrent cholesteatoma. In the remaining seven ears (53.8%) a canal wall down mastoidectomy was performed. Of all meatoplasty performed, seven (53.8%) evolved with stenosis of the external auditory canal.Our results suggest that most patients with EEC syndrome and middle ear cholesteatoma should be considered for a canal wall down mastoidectomy due to extensive disease and a high rate of recidivism. In addition, a high percentage of postoperative stenosis of the external auditory canal was found in this group.