Pachydermoperiostosis is a rare disorder characterized by the involvement of skin and bone, and in some cases it can have a mild adverse effect in the eyelid. Although the etiology is still unclear, idiopathic or hereditary cases, in an autossomal dominant inheritance, have been reported. This study is a case report of a patient with severe blepharoptosis due to pachydermoperiostosis, which describes the surgical procedure and the physiopathology of the condition.