Frontonasal dysplasia (FND) or craniofacial dysraphism includes a variety of craniofacial defects. FNDs are rarely associated with intracranial lipoma. The majority of intracranial lipomas are incidentally identified on radiological examinations. They are commonly accompanied by other congenital intracranial malformations. Moreover, they are rarely associated with subcutaneous lipoma. We present a rare case of midline craniofacial dysraphism with interhemispheric calcified lipoma together with subcutaneous lipoma and agenesis of the corpus callosum.