ectodermal dysplasia with natal teeth, Turnpenny type (Monarch Disease Ontology identifier MONDO_0011041) is characterized by hypo- or oligodontia and acanthosis nigricans. It has been described in four generations of one family. Onset generally occurs during adolescence. Some patients were born with multiple teeth. Hair anomalies (sparse body and scalp hair) were also reported. Inheritance is autosomal dominant.