Introduction: The first case of pediatric spindle cell / pleomorphic sarcoma associated with Haberland syndrome or Encephalocraniocutaneous Lipomatosis, a rare ectomesodermal dysgenesis defined by the triad that includes ocular, skin, and central nervous system involvement, which is usually unilateral, is described. This disorder is attributed to a postzygotic mutation responsible for dysgenesis of the neural tube and crest.

Clinical Case: We present the case of a 10-year-old boy, who evolves with developmental delay, motor deficit, intellectual deficit, and epilepsy, associated with spindle cell / pleomorphic sarcoma. We describe his clinical evolution, electroencephalography, and neuroimaging of him.

Conclusion: The hypothesis that Haberland syndrome is associated with an increased risk of tumor development is intriguing, although the rarity of the disease currently prevents us from drawing definitive conclusions about this possible link between the two entities.