A case report of classical presentation of rare melanotic neuroectodermal tumor of infancy

Melanotic neuroectodermal tumor of infancy (MNTI) is a rare neoplasm in infants, often mimicking aggressive malignancies or odontogenic lesions, particularly on maxillary or mandibular alveolar ridges. We report a 4-month-old male presenting with a one-month history of swelling in the right upper mouth, rapidly increasing over 15 days. Clinical examination showed a firm, bluish swelling on the right maxillary alveolus. Provisional diagnosis included rhabdomyosarcoma, with differential diagnoses of peripheral giant cell granuloma and small round cell tumor. Imaging revealed an expansile soft tissue mass in the right maxilla without systemic involvement. Surgical excision under general anesthesia was performed. Histopathology confirmed MNTI, supported by immunohistochemistry markers such as CK and HMB45 positivity. The patient showed no recurrence or complications over eight months of follow-up, with normal growth and healing in the affected region. This case underscores the importance of differentiating MNTI from malignancies in infants. Early diagnosis and surgical intervention are essential. A timely treatment and close follow-up ensures favorable outcomes in MNTI cases.