A clinical case of a 12-year-old girl involving agenesis of multiple permanent maxillary teeth, and presence of a full complement of permanent mandibular teeth, is reported. No such case is previously reported in literature. A prosthetic rehabilitation using a removable maxillary denture was performed to improve the facial profile, have satisfactory intercuspation of teeth, and restore masticatory function along with significant esthetic improvement in smile. Careful monitoring on a long-term basis, educating the parents about growth changes in the child and importance of replacing prostheses according to these changes, is desired.
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