Melanotic neuroectodermal tumor (MNET) of infants is a rare benign neoplasm detected mainly in children of the first year of life. Standard therapy consists of radical surgical removal of the tumor. Currently, the treatment of patients with recurrent and unresectable forms of the disease is relevant. The number of publications using polychemotherapy and radiation therapy is extremely limited. This article describes a clinical case of the occurrence of MNET in the anterior fontanel with a recurrent course. Proton radiation therapy was chosen as an alternative treatment method.