Rationale: Melanotic neuroectodermal tumour of infancy (MNTI) is universally described as a rare, benign, pigmented lesion which most frequently involves the maxilla. Its origin is well established to be in the neural crest cells. Due to the high recurrence rate and aggressive behaviour mimicking malignancy, it poses a great challenge in their diagnosis, treatment planning, and prognosis.
Patient concern: Two-year-old female with no known comorbidities was brought in with the chief complaint of a growing swelling in the upper lip region.
Diagnosis and treatment: She was taken up for resection of the tumour under general anaesthesia. The specimen was subjected to histological and immunological examination confirming the diagnosis of MNTI.
Outcome: The postoperative period was uneventful. After regular follow-up, the patient showed satisfactory healing with no signs of recurrence.
Take-away lessons: Based on our experience, we feel that the diagnosis of MNTI is mainly clinical. Early conservative surgical excision and regular follow-up provide an excellent result with good prognosis.
Keywords: Melanotic neuroectodermal tumour of infancy; neural crest; resection; vanillylmandelic acid.
Copyright: © 2022 Annals of Maxillofacial Surgery.