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The case described here refers to a female patient, black, 2-years-old, accompanied by her mother who reported the increase of hair in the external ear and face of her baby since birth. At 5 months, the thin, light blond lanugo hair covered the entire face, armpits, groin and lower limbs. The parent denied the presence of lanugo hair on the mucous membranes, palms and plants. The patient was born by cesarean delivery at term and had had normal psychomotor development, but with delayed dentition. The dental units available were deformed. The child's parents were second cousins. The mother denied the use of drugs or alcohol during pregnancy, as well as any family members with similar signs or symptoms. On examination, the patient had long thin and light-colored hair on the face, especially on the upper lip and mandibular regions, axillae, external genitalia, back and lower limbs ( and ). The presence of five abnormal dental units was observed. The patient, showing no other associated anomalies, was diagnosed with Congenital Hypertrichosis Lanuginosa, and referred for evaluation to the dentistry, genetics and ophthalmology clinics.