Bipolar disorder is seen comorbid with many disorders such as diabetes mellitus, thyroid disorders and Usher syndrome. Some research results and known mechanisms show bidirectional connections between affective disorder and some somatic disorders. Bietti dystrophy is an autosomal recessively inherited rare dystrophy, mostly seen at 20-40 age and decreasing visual acuity, night blindness and narrowing visual field symptoms are seen. The aim of this article is to present a case of Bipolar disorder comorbid with Bietti crystalline retinal dystrophy. Researches show that the CYP4V2 gene considered responsible for Bietti crystalline retinal dystrophy exists in human heart, brain, placenta, lung and liver and this gene transcript an active protein in fatty acid metabolism. It’s been shown that Bietti crystalline retinal dystrophy is an abnormal fatty acid metabolism disorder and the protein coded with CYP4V2 is a fatty acid hydroxylase enzyme. Our patient was a 26-year-old, primary school graduate, married male and living with his wife and daughter. He has been working as a cleaner since 8 years. Since last one month, suspiciousness, thoughts of follow up, infidelity, visual hallucinations, increase in the amount of speech and homicidal behaviors has been observed. He was interned. His visual problems were firstly started at secondary school years and by this reason, he had to leave the school. He was diagnosed as having Bietti crystalline retinal dystrophy, when he was 13 years old. He could not be in the army because of visual loss and he became more introverted and withdrawn. During this process, he had never been consulted to a psychiatrist, have no psychiatric family history. His uncle was diagnosed retinitis pigmentosa. His mood was dysphoric, his speech was accelerated, psychomotor activity increased. Jealous, persecutory and reference delusions were defined. Memory functions were normal, spontaneous attention was normal. His impulse control was decreased. He had no insight. According to DSM 5 diagnostic criteria, he was diagnosed with Bipolar Affective Disorder. Treatment of parenteral haloperidol 20 mg/day, biperidene 5 mg/day and oral olanzapine 10 mg/day was administered. After clinical observation, parenteral treatment was stopped and treatment of risperidone 4 mg/day, Olanzapine 10 mg/day and valproic acid 1000 mg/day was started. At 15th day of the treatment, oral risperidone treatment was changed with 4 mg/day due to increase in severity of jealousy delusions. During his clinical progress, his laboratory results were between normal ranges and electroencephalography and diffusion magnetic imaging results were reported normal. By presenting this case, we try to attract attention on the probable association between bipolar affective disorder and rarely seen Bietti’s crystalline retinal dystrophy.