Abstract: Frontonasal dysplasia (FND) is a rare syndrome characterized by malformations of the central portion of the face, especially of the forehead, nose, and philtrum. FND is associated with hypertelorism, a hidden encephalocele, and a cleft of the nose. Occasionally, affected individuals also experience abnormalities of the brain and craniofacial bones. In such cases, a frontal lipoma or calcification of the falx cerebri suggest the existence of a lipoma of the corpus callosum. We present the case of a male newborn with a congenital lipoma 15 mm in size located in the medial frontal line. Magnetic resonance imaging confirmed complete agenesis of the corpus callosum. While there is controversy about the association of frontal lipoma and lipoma of the corpus callosum and FND, we believe our case supports the concept that the changes are due to the same underlying pathogenic mechanism. Therefore, we recommend imaging of the central nervous system in newborns with a congenital lipoma located in the craniofacial midline.