Aim: To find out the extent of simple disease like keratosis obturans and possible complications it can give rise to. Background: Keratosis obturans was first properly described and named by Wreden of St. Petersburg in 1874, who differentiated the condition from that of impacted wax (which was then called ceruminosis obturans). Keratosis obturans is the accumulation of a large plug of desquamated keratin in the external auditory meatus. It is thought that keratosis obturans is due to abnormal epithelial migration of the ear canal skin. We are presenting a rare case of keratosis obturans with lateral semicircular canal fistula. Case description: A 56-year-old female presented with right ear discharge since 15 days and giddiness with vomiting since 5 days with past history of right ear discharge since 5 years. Clinical examination revealed keratin plug in right external auditory canal and posterior meatal wall bulge with dull tympanic membrane. Otoendoscopy shows wax with keratin debris and pulsatile discharge. Patient had horizontal nystagmus, gaze evoked with head shake test positive. Fistula sign was negative. High-resolution computed tomography (HRCT) temporal bone showed erosion of lateral semicircular canal and no ossicles. Modified radical mastoidectomy with type 4 tympanoplasty was done. Keratin debris with wax was seen in attic and aditus. Fistula closed using periosteum. Intraoperatively, patient developed facial nerve weakness which recovered postoperatively. Conclusion: We were able to treat the patient by prompt intervention and diagnosis of the condition resulting in adequate recovery of the patient. Clinical significance: Keratosis obturans is a rare disease, and the true incidence of this is not known with its natural history and progression. High clinical suspicion and prompt management are important as treatment delay results in serious complications.