Cholesteatoma of the paranasal sinuses is a rare condition. The purpose of this paper is to present a child with a congenital maxillary sinus cholesteatoma. An 18-month-old girl presented with a 4-week history of right cheek and intraoral swelling. Examination revealed a smooth swelling of the right hard palate in association with the facial swelling in the maxillary region. An inferior meatal antrostomy revealed pultaceous debris in the right maxillary antrum and biopsy confirmed a maxillary sinus cholesteatoma. The inferior meatal antrostomy was enlarged to allow exteriorisation of the disease. Recurrence of the disease has not presented on follow-up. An exteriorisation procedure as performed, in child of this age, allows normal facial growth. If recurrence develops then further treatment may be instituted in a more mature facial skeleton.