We present a clinical otoscopy image of the left ear revealing a homogenous whitish retro tympanic formation with an otherwise calm ear drum, without perforation nor retraction pocket in a 6-year-old male who was referred for progressive unilateral conductive hearing loss. Such findings are highly suggestive of congenital cholesteatoma and should warrant further investigation. A cone beam CT revealed a soft tissue density mass filling the left middle ear cavity and diffusion-weighted magnetic resonance imaging later confirmed the diagnosis with a focal restriction of diffusion. The child underwent surgery and a pearly white matrix distinctive for cholesteatoma was removed. Our aim is to depict a typical clinical finding which should immediately raise suspicion for an uncommon pediatric otologic pathology. Written consent for publication was obtained from the patient and his legal representative.