Several pathologic lesions can be responsible for rapid swelling of the maxilla during infancy. Two of these uncommon lesions are melanotic neuroectodermal tumor of infancy (MNTI) and myofibroma. Despite these lesions being benign, they can have a destructive behavior that needs a meticulous diagnosis and early intervention. Melanotic neuroectodermal tumor of infancy often presents as a fast-growing lesion, suggesting a clinical impression of infection or malignant neoplasm. Local excision and curettage are appropriate treatments for MNTI. Myofibromas are a rare lesion in the maxilla during infancy and sometimes are mistaken for malignant lesions. Surgical excision has been considered as a choice treatment for maxillomandibular lesions.