To date, an MS‐like illness has only been described in one patient with an OPA1 mutation,14 although another patient had coincidental MRI findings consistent with MS,15 and several case series have described abnormal white matter high signal changes in OPA1 mutation carriers.16, 17, 18 In this report, we describe three additional unrelated white Caucasian patients who presented to our tertiary neuro‐ophthalmological center with a clinically definite spinal cord MS phenotype, and who were eventually found to harbor pathogenic OPA1 mutations. Here, OPA1 is linked to myeloid sarcoma.