Pachydermoperiostosis is a rare genetic disease that causes major bone and skin changes. Severe ptosis is a prominent finding in this peculiar condition. We report here 2 patients with typical features of pachydermoperiostosis. Their tarsal plates were enlarged and infiltrated by sebaceous gland tissue. One patient also displayed diffuse intratarsal lacrimal gland hyperplasia. As far as we know, this is the first report of ectopic lacrimal tissue in pachydermoperiostosis. Tarsal and skin morphologic changes should be addressed during ptosis correction in these patients.