Abstract
Intracranial cholesteatoma is a rare entity most commonly present in the middle ear region. Due to this, a consensus on serial imaging and when to intervene is not well established. Here, we present a rare case of an HIV-positive patient without any prior history of trauma or infection who developed a T1 and T2 hyperintense lesion of the sphenoid wing, confirmed as cholesteatoma, that nearly doubled in size over six years, causing mass effect on the left orbit and ultimately requiring resection. To the best of our knowledge, this is the first reported case of sphenoid wing cholesteatoma without sinonasal/middle ear involvement in an HIV positive patient. Overall, we recommend that these lesions be followed with interval imaging and offered resection when rapid growth or mass effect is observed.