Objective To discuss the main points of diagnosis and differential diagnosis of retinoblastoma patients with non-leukocoria as main symptoms.Methods The retrospective study included 111 patients seen in Eye Center of Beijing Tong Ren Hospital between January 1990 and December 2006,whose main complaints were not leukocoria.The medical records were reviewed, and the clinical manifestations and histopathological findings were evaluated.Results These patients accounted for 19.2% of all patients during this period.The age of patients averaged 40.8 months and the complaints lasted for a mean time of 4.8 months.Over 50% of patients were equal to or older than 3 years old and a relatively large percentage (23.4%)of patients were equal to or older than 5 years.The main symptoms at diagnosis were decreased vision (40.5%),strabismus (27.9%), ocular redness and swelling with or without pain (21.6% ), enlarged eyeball (6.3%),photophobia and lacrimation (4.5%),atrophy of eyeball (3.6%), and others (11.7% ).Histopathological examination demonstrated that tumors were confined within the globe in 73.9% of patients and spread extraocularly in 26.1% ,and cut ends of the optic nerves were affected in 6 eyes (5.4%).Tumors with spontaneous regression were found in 5 cases and all were in intraocular stage and 4 of these occurred atrophy of eyeball.The differential diagnoses mainly included strabismus,orbital inflammation,intraocularinflammation,glaucoma,intraocular hemorrhage,other disease that caused poor vision.Conclusions In the clinic, children who display poor vision, strabismus, ocular redness and swelling, enlargement or atrophy of the eyeball, intraocular hemorrhage ,photophobia and lacrimation with unidentified reason or with repeated attack should be noticed and considered the possibility of retinoblastoma.