<p>During otosclerosis surgery, it is common practice for the surgeon to cut the stapedius tendon. However, the congenital absence of the stapedius muscle and its tendon is a rare anomaly, documented in only nine indexed cases involving live patients. This condition results from complex embryologic sequences where the internal portion of the interhyale- the mesenchymal condensation separating the cranial mesenchyme of the second pharyngeal arch (stapedial anlage) from Reichert’s cartilage- remains either absent or undergoes regression. Patients typically present with non-progressive, occasionally bilateral conductive deafness, although this cannot be reliably confirmed through Pure Tone Audiometry (PTA) and High-Resolution Computed Tomography (HRCT). Consequently, it is one of the least probable differential diagnoses of conductive deafness with an unremarkable tympanic membrane, with definitive diagnosis made intraoperatively. This rare condition often coexists with other genetic or acquired disorders of the middle and inner ear, such as otosclerosis and tympanosclerosis. In this study, we present the case of a 36-year-old female with gradually progressive bilateral conductive hearing loss, more pronounced in the left ear. During left exploratory tympanotomy, we discovered the absence of the stapedius muscle and tendon, the pyramidal process, and the chorda tympani, along with evidence of otosclerosis. The stapes footplate was fixed, and the round window reflex was absent. A stapedotomy with piston insertion was successfully performed. Awareness of such variations or anomalies of the stapedius muscle and tendon is crucial for surgeons operating within the tympanic cavity, particularly during otosclerosis surgery. This case underscores the importance of considering these noteworthy anomalies to avoid intraoperative surprises and ensure optimal surgical outcomes.</p>