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Enamel may be found ectopically as enamel pearls (EPs), which are frequently associated with advanced localized periodontal (LP) destruction. This study presents a case in which an unusual non-tooth-related enamel pearl and three teeth-related enamel pearls with LP disease were found and treated without teeth extractions. A 47-year-old female patient presented at the New York University College of Dentistry with four EPs, three of which were associated with periodontal pockets and/or bleeding on probing (BOP), and one of them not related to any tooth. Periodontal therapy included scaling and root planing and open flap debridement with removal of the EPs. Two pearls were histologically analyzed by polarizing microscopy and scanning electron microscope (SEM). Clinical periodontal parameters, including probing depth and BOP, were measured. All enamel found in the pearls had the same general morphologic appearance when examined by a SEM. The non-tooth-related pearl could be classified as Rodriguez Ponte "adamantinoidea" pearl. Probing depths at 3 months and 9 months after EP removal diminished considerably. Only after the treatment was there no BOP. Before treatment it was in some areas, as shown in Table 1. The LP related to the EP was resolved. The authors conclude that early recognition of enamel pearls is important in the prevention of periodontal destruction, and removal of EPs by a surgical approach as an adjunct to mechanical periodontal treatment resulted in resolution of the LP. The authors state that this is the first time an "adamantinoideas" pearl is being reported on clinically in the literature.