Objective: To observe and analyze the clinical features and misdiagnosis of iridocorneal endothelial  (ICE) syndrome. Methods: In this retrospective case analysis, 86 eyes of 86 patients who were diagnosed  with ICE syndrome between 1993 and 2015 in Qingdao Eye Hospital. Sex, age, maincomplaints, course  of disease, types, complication and misdiagnosis were recorded and analyzed. Results: Eighty-six cases  were unilateral. The ratio of male to female was 1:1.2, and the age ranged from 20 to 73 years, average  50.1±11.8 years. 91% of patients with progressive visual loss and red eye were the main complaints. The course of disease ranged from 0.17 to 10 years, average 2.4±2.3 years. 1% of patients complained about  pupil deformation, the course of disease was 1 year. 6% of patients complained black eye foggy, the  course of disease ranged from 0.5 to 2 years, average 1.4±0.6 years. 2% of patients with high intraocular  pressure founding by physical examination were treated in time. 95% of patients with obvious iris atrophy  were classified as primary progressive iris atrophy, Chandler and Cogan-Reese syndrome were 4% and  1%. The typical morphology of corneal endothelial cell showed irregular, cavitation, central black area  and brighter edge. The atypical sign was no significant iris atrophy. 68% of patients had developed  glaucoma and corneal endothelial dysfunction. Ultrasawnd biomicroscepy and angle mirror examination  showed peripheral iris extensive anterior and local filamentous adhesion, angle pigment classification  level was 1 to 3. 27% of patients were misdiagnosed for the first visit and 20% of these patients were  misdiagnosed in other hospitals. 8% of patients were misdiagnosed as primary glaucoma, 12% of  patients were misdiagnosed as Posner-Schlossman syndrome, Fuchs syndrome and uveitis. Absence of  corneal endothelial cell morphology examination and atypical clinical manifestation were main causes of  misdiagnosis. Conclusions: Progressive visual loss is the main reason for patients with ICE syndrome,  long course of disease and delay treatment. More than 50% of patients have complications at visiting time.  95% of patients are primary progressive iris atrophy. Most easily misdiagnosed as primary glaucoma and  uveitis. Absence of corneal endothelial cell morphology examination and atypical clinical manifestation are  main causes of misdiagnosis.