Congenital granular cell tumour (CGCT) is a benign lesion that predominantly arises from the alveolar ridges of neonates, especially the maxilla. However, it's only 10 percent of multiple lesions in all reported cases, in which simultaneously mandibular and maxillary involvements are more extremely rare.For treatments of multiple CGCTs, few standard procedures were reported. In addition to surgical excision, which refers to a preferred method, conservative treatment is an available choice. Here, a case of multiple CGCTs using different therapeutic strategies was reported because of its rarity and innovation. A fiveday-old female newborn presented two congenital masses attached to the right mandibular and maxillary alveolar ridge. The size of the mandibular lesion causing difficulty in feeding was 3 cm in diameter and 0.5 cm in the maxilla. Based on different manifestations, surgical excision and conservative treatment were adopted respectively. The mandibular mass was excised while that in the maxilla underwent spontaneous regression. Satisfactory results were achieved for this patient. There was no evidence of recurrence after a 6-month follow-up. Microscopic examination and immunohistochemistry analysis confirmed the diagnosis and differential diagnosis of CGCT and even proposed the possibility of histogenesis from neural crest. Moreover, we reviewed the literature and summarized the characteristics to provide new ideas for the treatment of multiple CGCTs.