The authors report a case of a congenital dermoid inclusion cyst of the region of the anterior fontanel, in a white boy, 6 months of age. The child had a normal clinical examination as well as normal milestones development. The skull X-Rays showed a soft tissue mass at the region of the anterior fontanel. The underlying bone was not eroded. During the operation, one could observe a cystic mass, well encapsulated, with moderately thickened walls and a small amount of a clear, colorless fluid in it. There was no fistulous tract between the cyst and the skin or the intracranial cavity. The histopathologic examination of the specimen showed a cystic structure surrounded by a stratified squamous epithelium and a few adnexal structures in it. The authors make an extensive review of the literature on the subject. They have found 135 cases published until 1984.