Background: Classification schemes are useful in the formulation of di
fferential diagnoses. Thoughtful commentary has been devoted to the cl
assification of blepharoptosis, but the causes of eyelid retraction ha
ve received less attention in published reports. Although eyelid retra
ction most frequently is associated with Graves ophthalmopathy, numero
us other entities may cause the sign. This study was undertaken to pro
vide a more comprehensive differential diagnosis and classification of
eyelid retraction. Methods: A series of patients with eyelid retracti
on was studied, and pertinent published reports were reviewed. Results
: Forty-four patients with different causes for eyelid retraction are
described. Normal thyroid function and regulation were confirmed in al
l patients in whom Graves ophthalmopathy could not be excluded by clin
ical, biochemical, or historical criteria. Conclusion: Based on a seri
es of patients and reported cases, a differential diagnosis for eyelid
retraction is proposed using a classification system comprising three
categories (neurogenic, myogenic, and mechanistic).