Melanotic neuroectodermal tumor of infancy in ovary

Rationale: Melanotic neuroectodermal tumor of infancy (MNTI) is an extremely rare benign pigmented neoplasm of neural crest origin with rapid expansile growth and a high recurrence rate. It is predominantly found in infants of <1 year of age, involvement of the head-and-neck region is the most common presentation though it is reported at other sites including mediastinum, shoulder, thigh, foot, epididymis, uterus and ovary. The patient reported here is the third case of MNTI presenting in an ovary, and the first reported in the infant ovary. Patient concerns: A 33-month-old girl was presented to our unit for a huge abdominal mass. Diagnosis: MNTI was eventually diagnosed by histological manifestations supplemented with immunohistochemical findings. Interventions: Exploratory laparotomy and complete resection were conducted successfully. Outcomes: Postoperative course was uneventful and no recurrence was displayed in the 6-month follow-up. Lessons: This case emphasizes that pediatric surgeons and pathologists must always consider the possibility of MNTI while dealing with ovarian neoplasms in infants. Although considered to be a benign tumor, proper treatment and close clinicoradiological follow-up of this tumor are of great importance