Osteomas of the middle ear are rare benign tumors. They commonly arise from the promontory. To date, a total of three cases of osteomas of the ossicles, inseparable from malleus and incus, have been described in the English literature. Among them, only one case osteoma with congenital cholesteatoma has been reported. Recently a 6-year-old boy with osteoma associated with congenital cholesteatoma presented to our service. The osteoma and congenital cholesteatoma were removed successfully by canal wall up mastoidectomy with staging operation. A rock-hard mass was separated from the footplate. The malleus and short process of incus were intact. Histopathological examination confirmed the presence of the osteoma.