Introduction A cholesteatoma is a three-dimensional sac lined by keratinized squamous epithelium containing desquamated keratinized epithelial cell which secretes enzymes that have the tendency to expand and erode the bony structure underlying it and cause intracranial and extracranial complications. This cystic mass is in an abnormal location such as the middle ear, the petrous apex, or the external auditory canal (EAC). It is mostly found in the middle ear and rarely in the EAC. Here we have reported a rare case of unilateral primary EAC cholesteatoma with mild hearing loss in a middle-aged male. Case Report We have reported a case of a 34-year-old male with complaints of right-sided ear discharge and right-sided decreased hearing for the last 5 to 6 years. On examination, right ear EAC was found to be dry, and a sac was observed in posterior wall extending to mastoid present with clear attic, and intact retracted tympanic membrane that was then followed by radiological evaluation to establish the diagnosis of EAC cholesteatoma. This was surgically treated and ear was cleared of all disease. Patient's symptoms improved postoperatively. Conclusion Primary EAC cholesteatoma with disease-free middle ear is a rare finding and there is very less definitive literature available on the pathogenesis of the same.