Congenital cholesteatoma is a rare entity that constitutes for only 2-4 percent pholesteatoma in children. This is a squamous cell pseudotumor derived from ectodermal residue in middle ear. Late detection may result in ossicular chain erosion, hearing loss and intracranial complications. the authors reported a case with congenital middle ear cholesteatoma in infant found at Vietnam - Cuba hospital, Hanoi. This was a three year-old infant who admitted to hospital for nasal obstruction, rhinorrhea and earache. Endoscopy revealed a white mass behind an intact tympanic membrane at superio-anterior quadrant. CT scan showed a round, opaque mass (2-3 mm) in the tympanic cavity anterior to manubrium. Those findings were consistent with congenital cholesteatoma stage 1 The child underwent transcanal endoscopic surgery for cholesteatoma removal. Pathologic characteristics confirmed the diagnosis of congenital cholesteatoma.