A patient with the rare condition of pachydermoperiostosis with secondary ptosis and floppy eyelid was successfully treated with a combination of levator advancement and an upper eyelid tarsal strip. This is the second report in the literature of combined floppy eyelid and ptosis with this condition and the first report of this surgical approach for its management. The histopathology of the excised eyelid tissue found changes consistent with both pachydermoperiostosis and floppy eyelid syndrome.