&NA;We report a 2‐year‐old male with aplasia cutis congenita of the scalp, epibulbar dermoids, strabismus and macrocephaly. In our opinion, he is affected by the Oculo‐Ectodermal syndrome first described by Torielloet al.(1993).Am J Med Genet45:764‐766]. This is the sixth report of patients with this rare entity. Our case further expands the clinical spectrum of the syndrome to include mental retardation, seizures and microscopic hair changes.
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