Enamel pearls are enamel anomalies on primary and permanent teeth roots that usually appear at furcation areas, especially in maxillary second and third molars. Enamel pearls usually occur singularly, but as many as four have been observed on the same tooth. This report describes an unusual case of multiple enamel pearls associated with periodontal pockets localized on all maxillary first and second molars. Because the patient had an advanced stage of periodontitis, the maxillary right first and left second molars were extracted. The remaining two maxillary molars were included in a strict follow-up protocol. Enamel pearls were confirmed as the cause of localized periodontitis; therefore, it is very important to recognize their radiologic aspect to ensure proper treatment of the involved teeth.