Objective: To investigate the association between aortic dissection (AD) and functional adrenal tumors. Methods: Two patients with AD and concomitant functional adrenal tumors admitted in recent years were reviewed, and the relationship between the two diseases was analyzed and combined with related literature review. Results: One patient refused surgical treatment, while the other underwent first-stage aortic dissection endovascular repair and second-stage laparoscopic adrenal tumor resection, and recovered after surgery. There were a total of 9 cases with AD and concomitant functional adrenal tumors reported in English literature, and only 2 relevant cases seen in domestic literature. Relationship analysis between the two diseases indicated that the hormones secreted by the functional adrenal tumors may induce the AD formation through secondary hypertension or their direct actions. Conclusion: AD associated with functional adrenal tumors is rare and there may be a connection between the two diseases so, in clinical practice, rigid screening should be performed.