Background. Acute confusional migraine (ACM) is a rare variant of migraine, mainly prevalent in children and adolescents. It is not currently indexed as a distinct variant of migraine likely since only a few cases were reported in the adult population. We report a case of delayed ACM diagnosis in a young man and present a concise-related literature review. Case Presentation. A thirty-eight-year-old man with a past medical history of migraine, not on any treatment, presented with headaches accompanied by confusion. Over a two-year period before the current presentation, he experienced two episodes of confusion, which required hospital admission for evaluation: once mislabeled as a psychiatric illness and diagnosed as a migrainous infarct in the second hospitalization. In the current presentation, he reported a similar history of headache accompanied by confusion. The examination was remarkable for disorientation; otherwise, no focal deficit was elicited. Laboratory testing, cerebrospinal fluid, and neurological imaging were all unremarkable. His symptoms improved spontaneously within less than twenty-four hours, similar to his previous presentations. After two-year history of episodic confusion and after excluding other plausible causes of confusion, guided by proposed diagnostic criteria, we diagnosed him as a case of ACM. The patient remains well at the follow-up of two months after discharge. Discussion and Conclusion. ACM is a rare variant of migraine and is often a challenge for clinicians to diagnose appropriately. Until recent years, the disease was thought to be limited to children and adolescents. However, recently few reports also expanded the incidence of this entity to the adult population. There is a significant gap in knowledge about proper identification and treatment of this condition, leading to delayed or overlooked ACM diagnosis. Moreover, the recent edition of the International Classification of Headache Disorders (ICHD-3) does not account for this entity, thereby further adding to physicians' lack of awareness regarding this migraine subtype. The authors emphasize that clinicians be aware of this entity and adequately utilize the existing proposed diagnostic criteria for ACM until standardized and validated tools are available. We also believe that this entity should be acknowledged in the subsequent migraine guidelines and classifications.