Cholesteatoma is a mass of keratin debris in the middle ear cavity or mastoid. Congenital cholesteatomas may remain asymptomatic for many years and present during adulthood or may even be an incidental finding. We hereby describe a case of 41-year male with an isolated mastoid congenital cholesteatoma with sigmoid plate erosion and posterior cranial dura exposure extending into digastric muscle. Tympanomastoid exploration with sigmoid sinus resurfacing with hydroxyapatite granules was done. However, the case got complicated by extrusion of granules which was then successfully managed. This case report throws light on diagnosis and surgical management of isolated mastoid cholesteatoma. From this misadventure, we learnt that a successful mastoid cavity obliteration (using hydroxyapatite granules) in a canal wall up mastoidectomy can be achieved by providing hermetic seal.
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