Congenital melanotic tumors are among the very rare tumors of early childhood. Their histogenesis and biological significance are still controversial today. The ideas of an odontogenic origin and of a neuroectodermal pathogenesis of these tumors range among the most frequently advocated hypotheses. Until about 1970 this type of tumor was almost always referred to as benign. Meanwhile there has been a change in the assessment of the character of congenital melanotic tumors. Above all, this is due to the fact that malignant cases have been repeatedly reported since 1970. Apart from an extensive review of the literature, 2 own cases are described which were treated surgically. The histological evaluation of the tumor material supports the assumption that these tumors are of odontogenic origin.