Purpose: To study the natural course of hydroxychloroquine (HCQ) retinopathy, and to discuss the relationship between HCQ and its toxicity in retina in terms of ATP-binding cassette transporter gene, ABCR (also known as ABCA4) and HCQ blood level. Methods: Here we reported two cases of retinopathy associated with HCQ from a tertiary referral center. We then performed a literature review of studies on HCQ cessation in patients with HCQ retinopathy. Results: One of them stopped the HCQ therapy in her early stage of HCQ retinopathy and gained some regression on visual field testing. The other patient continued to use HCQ without screening till a severe toxicity in macula was found. Although she ceased HCQ, aggravation was found. Their HCQ blood level was 20557.4ng/ml and 402.8ng/ml respectively when they were advised to stop the treatment. Patient 1 was carrier of transition 6764G > T and patient 2 was heterozygous for the transition 5844A > G and 5814A > G . Conclusion : The study suggests that the integrity of outer retinal layers on OCT as a remarkable sign of possibility of recovery. But severe damage to macular structure can hardly regress which emphasizes the importance of HCQ cessation at an early stage of this disease. To the future, more studies are needed to better understand the relationship between HCQ retinopathy and ABCA4 genotypes and blood concentration.