Cockayne's syndrome (CS) is a rare, autosomal recessive disease resembling progeria. The features of CS do not appear until 4 to 5 years of age. Most patient presents with cachectic dwarfism, cutaneous photosensitivity, loss of adipose tissue, mental retardation, skeletal and neurological abnormalities, similar to the current case. The additional feature observed in the present case was actinic chelitis. We report a case of Cockayne‟s syndrome with pronounced oral manifestations and an unusual feature of actinic chelitis.

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Drainage