Background: Epidermal inclusion cysts very rarely occur in the midline in the area of the anterior fontanelle.
Case description: We report the second case of epidermal inclusion cysts in the midline in the area of the anterior fontanelle in world literature of a 7-month female infant who presented with a slow-growing soft mass located in the midline over the vertex of the head.
Conclusion: These lesions with clear contents can have all the features of encephalocele on clinical examination, and imaging will show the exact location, but histopathology will confirm the diagnosis.