A case of adenoma of nonpigmented ciliary epithelium (ANPCE) was reported and relevant literatures were reviewed. The left eye visual acuity of the patient gradually decreased for 3 months, and half a month was blurred vision. The vision examination and ultrasound biomicroscopy (UBM) from the left eye examination revealed a bulge in the peripheral iris in the left eye, with the boundaries are clear. The left eye was treated with ciliary mass resections and routine pathological examination: microscopy showed that the tumor tissue consists of well-differentiated epithelial cells, the tumor cells were arranged in tubes and cords, between the cells were seen red-stained unstructured basement membrane; immunohistochemistry showed: S-100 (+), Vimentin (+), EMA (+), CKpan (+), Melan-A (+); the final pathological diagnosis was ANPCE. There was no progression of the disease during the 3 months following the surgery on the follow-up date.