Background: Enamel pearls are developmental anomalies consisting of ectopic enamel, with or without dentine and pulp tissue. They most commonly occur on maxillary second and third molars and are less frequently associated with mandibular third molars. Although they may have periodontal implications, their anatomical location can also influence surgical management. Case report: A 32-year-old female was referred with recurrent episodes of pain and inflammation associated with the mandibular third molars. Both lower third molars were partially erupted and mesioangular, with no active pericoronitis or periodontal pathology at presentation. An orthopantomogram demonstrated an intimate relationship between both mandibular third molars and the inferior dental canals. Subsequent cone beam computed tomography (CBCT) demonstrated three convergent roots associated with the mandibular right third molar and an enamel pearl arising from the mesial root and extending lingually around the middle third of the root. The inferior dental canal demonstrated pronounced narrowing and deviation around the tooth, with the enamel pearl extending into the canal and towards the lingual cortical plate. The mandibular left third molar also demonstrated an intimate relationship with the inferior dental canal. Management and outcome: The increased risk of inferior alveolar nerve injury associated with surgical removal was discussed alongside the options of observation, coronectomy and complete extraction. Following informed discussion, the patient elected to monitor the mandibular third molars. The carious maxillary third molars were subsequently removed under local anaesthesia. Conclusion: This case highlights an unusual enamel pearl associated with a mandibular third molar and its potential implications for third molar surgery. CBCT provided important three-dimensional information regarding the relationship between the enamel pearl, tooth roots and inferior dental canal, facilitating risk assessment, treatment planning and informed patient decision-making.