Purpose: Two cases of ectodermal dysplasia with ocular manifestations are presented.Case presentation and results: The first case was a young girl with mild ocular involvement presented as dry eye and blepharitis. The second case was a 30 year-old man who presented as right corneal perforation due to severe dry eye. Dry eye in the first case was controlled with lubricants, along with treatment of blepharitis and finally punctual occlusion. Tectonic corneal graft with lateral blepharorrhaphy was performed in the second case.Conclusion: Ocular involvement in ectodermal dysplasia can present inself in a wide form. Appropriate treatment, control of dry eye blepharitis, especially in severe cases can improve ocular surface condition and preserve the eye.