PURPOSE:: To report a case of bullʼs eye maculopathy associated with mutations in RDS/PRPH2 and ROM-1 genes. METHODS:: We present a case report of a patient with a characteristic maculopathy and describe the multimodal retinal imaging findings including spectral domain optical coherence tomography and fundus autofluorescence and full-field electrophysiology. The results of genetic testing are also reported. RESULTS:: A 60-year-old woman presented with decreased vision and a remarkable bullʼs eye maculopathy with retinal examination. Fundus autofluorescence illustrated a striking pattern of speckled hyperautofluorescence and hypoautofluorescence that highlighted the bullʼs eye maculopathy in each eye and guided genetic testing, which confirmed a mutation of the RDS/PRPH2 gene and a novel mutation of the ROM-1 gene. CONCLUSION:: Multimodal imaging including fundus autofluorescence may guide genetic testing in patients with a characteristic maculopathy. RDS/PRPH2 genetic mutation can be associated with a bullʼs eye maculopathy with a signature fundus autofluorescence presentation.