Melanotic neuroectodermal tumors of infancy are rare neoplasms that occasionally may involve the skull. Usually such lesions present as an anterior fontanelle mass that grows rapidly. Very rarely, skull lesions are located at different sites and have an indolent course, thus making the diagnosis more difficult. To illustrate this latter presentation, we present a case of a slowly growing melanotic neuroectodermal tumor of infancy located on the right lambdoid suture.