Melanotic neuroectodermal tumour of infancy and Aggressive desmoid fibromatosis are histologically benign tumors but with aggressive behavior, it’s occurring during the little infancy and are caracterised by Fast-growing, osteolysis and recurrence. The treatment is essentially surgical. Melanotic neuroectodermal tumour of infancy is a rare tumour which affects young children arising from neural crest with a black coloring. It grows rapidly and first touches the maxilla. We related the case of a three-month age male who presents a tumor in the right maxilla. The case has CT scan and histology feature. Aggressive desmoid fibromatosis is a benign tumor with locally infiltrative behavior and tendency to recur. We report a rare case involving the mandible arch of three years old girl with histopathological, immunohistochemical and imaging features. She underwent a large surgically resection of the arch of the mandible, there has been no recurrence three years after.