Simpson-Golabi-Behmel syndrome is an x-linked recessive overgrowth disorder characterized by macrosomia, mental deficits, a large head, prominent cranial sutures, midface deficits, hypertelorism, a broad nose, a wide mouth, macroglossia, dental malocclusions, a strongly arched palate, and musculoskeletal and limb abnormalities. The male sex is more commonly affected, with only about 200 cases being described. The presented case of a 2-year-old patient showed a flat facial profile, macroglossia, a deep tongue position with forced mouth closure, a large upper and lower jaw, and enamel formation disturbances at the primary molars. Preventive, interdisciplinary care of the patient regarding his oral health as well as dentition and speech development are recommended.