AIM:In order to investigate the features of fundus fluorescein angiography(FFA) in congenital hypertrophy of the retinal pigment epithelium (CHRPE) in patients with familial adenomatous polyposis(FAP). METHODS: Prospective FFA and ophthalmoscopy were carried out in 22 patients with 65 CHPRE lesions.RESULTS: A total of 86% were over half the optic disc diameter in size. About 74% of CHRPE lesions were close to retinal vessels. The retinal vascular changes were: with capillary non-perfusion area (46%), partly block of retinal vessels (8%), chorioretinal anastomoses (6%), capillary microaneurysms (3%), choriocapillary vessels can be observed (5%) and in the depigmented marginal halo (20%). About 10% of the lesions were not seen on ophthalmoscope and could only be detected by angiography.CONCLUSION: Even though the diagnosis of CHRPE is clinical, FFA may be useful in confirming the diagnosis, as well as detecting additional lesions not seen by means of ophthalmoscopic examination.