Atypical fibromuscular dysplasia (FMD) is an underdiagnosed cause of ischemic stroke. The typical angiographic pattern of a septum on the posterolateral side of a carotid megabulb is highly suggestive of atypical FMD. We report here on two patients with this highly suggestive pattern of carotid atypical FMD, but which histological examination revealed to be atheromatous lesions. Interestingly, contrast-enhanced ultrasound of the carotid artery showed enhancement of the spur, which should never be the case with an FMD lesion, which has no vasa vasorum. Our findings suggest that the results of studies reporting stenting of atypical FMD in cervical arteries should be interpreted with caution.