Riga-Fede syndrome is characterised as an ulcerative lesion that originates on the ventral surface of a neonate’s tongue due to vigorous back and forth movements of the tongue over the precociously erupted mandibular anterior teeth. This condition has been receiving discernible attention as a progressing condition with escalating case reports over the years. The aetiology of this issue is not precisely deduced; however, there have been multiple treatment options discovered that have secured success with higher precision. The purpose of this research paper is to exhibit various case reports and aetiologies responsible for this disease, as well as an explanation of different treatment plans depending on the status of the tooth, that is, the mobility of the tooth and the precarious chances of aspiration. Moreover, radiographic study to visualise if it is a supernumerary or a primary regular tooth and if it is associated with hindrance in breast-feeding causing soft tissue injuries and the state of the child’s health. A 28-day-old female infant was presented to the oral pathology clinic with a natal tooth exhibiting grade 2 mobility along with a lesion on the ventral surface of the tongue, leading to the clinical diagnosis of Riga-Fede disease. The treatment performed, consisting of the extraction of natal teeth, was selected as the treatment of choice over more conservative treatments for the rapid resolution of the lesion and the limited risk of inadequate nutrient intake since the tooth was interfering with the feeding process. Following the extraction of the tooth, it underwent microscopic studies to identify diverse changes associated with the erupted tooth. There was regular monitoring of the patient, and complete wound healing was observed after 4 weeks. The proposed treatment was successful, and the patient is still in follow-up without recurrence of the lesion after one year. This paper presents a concise review of the literature about neonatal teeth and their role in Riga-Fede disease.