Tehran University of Medical Sciences Basic & Clinical Cancer Research 2228-6527 14 3 2023 09 30 168 173 Jitendra Hazarey MBBS MS, MCh Shilpa Hazare MBBS, MS, DCH, DNB Shilpa Hazare MBBS, MS, DCH, DNB Isha Hazarey Jawaharlal Nehru Medical College, Sawangi, Wardha Pritish Bhansali Resident Doctor at Dept. of Pediatric, Vishal Mahant MBBS, DCH Consultant at Dept. Of Pediatrics Rashiqua Sadiya BAMS Resident Doctor at Dept. of Pediatrics, 2022 09 20 2023 05 13 Abstract- Melanotic neuroectodermal tumor of infancy (MNTI) is a rare condition that typically presents within the first 6 months of life. It rarely metastasizes but is locally aggressive. Melanotic neuroectodermal tumor of infancy has been reported under several other names including retinal anlage tumor and melanotic progonoma. Most commonly, melanotic neuroectodermal tumor of infancy originates in the maxilla. Tumor in previously reported cases are known to originate from different structures. Recommended treatment of MNTI consists of complete excision with wide tissue margins, although success has been reported after incomplete resection too. For those patients in whom surgical resection is not a preferred option, various chemotherapy approaches have been reported with mixed results ranging from failure to sustained tumor regression. In the present case report we have case of MNTI in one year infant which is successfully treated with subtotal excision. https://bccr.tums.ac.ir/index.php/bccrj/article/view/448