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The Nasopalatine Duct Cyst (NPDC) also commonly referred to as the anterior midline palatine cyst is a developmental cyst derived from the proliferation of embryonic epithelial remnants of nasopalatine duct. It is unique for the reason that it develops in only one location, which is the midline of anterior maxilla in close relationship to the nasopalatine canal. Radiographically, it presents as a well-demarcated ovoid or heart shaped cystic structure which is seen in close proximity to the apex of maxillary central incisors. Due to its anatomical location, it can be sometimes misdiagnosed as central giant cell granuloma, periapical cyst or follicular cyst. Chronic, large periapical lesions following traumatic injuries are challenging to diagnose and treat. The present case report described the successful management of a rare case of bilateral NPDC in a 30-year-old male patient, who gave a presentation closely mimicking that of an infected periapical cyst. The case was treated by both conventional endodontic therapy and surgical intervention. The NPDC tends to show a predilection in giving rise to squamous cell carcinomas which commonly occur in the anterior part of the maxilla. Therefore, cystic lesions seen in the premaxillary region require a thorough analysis so as to successfully treat the lesion and to avoid further complications that may arise due to a missed diagnosis.