Potentially life threatening when they rupture, hepatic artery aneurysms (HAAs) are uncommon and diagnosed at the fifth to sixth decade of life. HAAs are the second most common splanchnic aneurysms after the ones that involve the splenic artery and are more predominant in men than in women with a ratio of 3 to 2. Usually, HAAs are associated with inflammation or traumas to the arteries of the liver. Among the former group, atherosclerosis, arthritis, and collagen vascular disease are the most common, whereas liver biopsy or other radiologic interventions (ie, percutaneous abscess drainage) are frequent iatrogenic causes. HAAs are discovered incidentally in most cases, but some patients can present with intraperitoneal bleeding and hypovolemic shock. The etiology of prune belly syndrome (PBS) is poorly understood, but new evidence suggests that it is caused by mesodermal delay during the fetal development because of an intrauterine injury. We present a case of HAA in a young individual affected by PBS. To our knowledge, this report is the first to describe the existence of these 2 rare conditions in a single patient. We hypothesize that the mesodermal abnormality that involves PBS might be a rare cause of HAA.