Congenital anomalies of the ossicular chain are a rare condition in clinical practice. In literature described different variants of the ossicular chain anomaly from stapes footplate ankylosis to complete aplasia of the ossicular chain, oval, and round window. Possible congenital ossicular chain anomaly should be suspected in case of non-progressive conductive hearing loss with a normal ear drum during an otoscopic examination, without a history of previous ear infections or trauma. In this article, we described the clinical case of congenital ossicular chain anomaly presented as fixed incus. Stapes was presented as single, straight, broad crus that were attached to the central part of the footplate. Stapedius tendon was absent. The footplate was completely mobile. Exploratory tympanotomy was performed to confirm a diagnosis of congenital ossicular chain anomaly. The ossicular chain was reconstructed with PORP titanium prosthesis. Furthermore, a literature review was done, conclusions presented.