Intrinsic tooth stains are more permanent in nature than extrinsic stains and can be divided into two types systemic and local. Erythrodontia is reddish-brown or reddish-black discoloration of teeth caused due to congenital erythropoietic porphyria (CEP) or Gunther disease. It is the rarest type of porphyria and is usually perceived in infancy. An 8-year-old girl with erythrodontia was referred to a private clinic in the United Arab Emirates with a chief complaint of discolored primary teeth with a medical history of CEP and severe right sensorineural hearing loss. A complete esthetic rehabilitation was done.
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