Objective To investigate the clinical manifestations and possible reasons of the missed and inaccurate diagnosis of Fuchs syndrome. Methods The clinical data of 85 patients with Fuchs syndrome who were diagnosed and treated from June 1999 to December 2003 were retrospectively analyzed. The disease history of each patient was carefully recorded. Slit-lamp microscopy was performed on the patients. The character and distribution of keratic precipitates (KP), color of the iris, depigmentation, and complications were noted in detail. The data recorded in other hospitals were analyzed and the reasons of missed and inaccurate diagnosis were statistically analyzed. Results The age of the patients ranged from 13 to 72 years, and binocular involvement was noted in 76 patients. None of the patients showed ciliary congestion and iris synechiae. Middle-sized or stellated KP was found. Triangle-distributed KP was seen in 13 patients, and diffuse distribution behind the corneal or in the pupil area was in 72. Anterior-chamber flare was observed in all of the patients, while anterior-chamber cells were only noted in 42 patients. The iris with different degrees of depigmentation was found in all the patients. Complicated cataract and increased intraocular pressure occured in 44 and 19 patients, respectively. The diagnosis in other hospitals mainly included uveitis, anterior uveitis and complicated cataract. Conclusions Fuchs syndrome is characterized by depigmented iris and typical KP. Missed and inaccurate diagnosis is mainly due to the unawareness of its clinical features. (Chin J Ocul Fundus Dis, 2005, 21: 360-362)