&NA;A new case of Goltz's syndrome, focal dermal hypoplasia, is documented. The literature is reviewed. The composite individuality warrants considering Goltz's syndrome a separate syndrome in the ectodermal dysplasias. Caution against diagnosing “condyloma‐like” lesions occurring in ectodermally dysplastic patients as condyloma acuminatum is urged. Neoplastic transformation and the tendency to recurrence after surgical excision of the cutaneous angiofibromas pose a difficult problem in surgical management.