Spontaneous cholesteatoma of the external auditory canal (EAC) is an uncommon condition that is difficult to diagnose. In a patient with such a possibility, serious clinical investigation along with radiologic and histologic exploration should be performed early on because a delay in treatment can lead to severe complications. Given the rarity of EAC cholesteatoma, no therapeutic consensus has emerged. The type of management depends on the extensiveness of invasion and bone erosion and the status of the neighboring structures. The primary therapeutic objectives are to eradicate the cholesteatoma and then to fill in the residual cavity, which in our opinion can be best accomplished with a muscle flap and EAC reconstruction. Postoperative follow-up should be carried out to look for infections, stenosis, and recurrence. We report a new case of spontaneous EAC cholesteatoma, and we review its diagnostic and therapeutic challenges.