Posterior circumflex humeral artery (PCHA) aneurysm formation and thrombosis as overuse injury in the dominant shoulder can result in ischemia of the forearm, hand and digits due to arterial embolisms. Increased awareness among physicians is necessary as PCHA pathology (PCHAP) might be underdiagnosed. Knowledge of sports and professions at risk and risk-factors for obtaining PCHAP is essential for the development of future preventive measures. This study aims to identify sports and professions at risk as well as risk factors for PCHAP. The databases of MEDLINE, EMBASE, BIOSIS, CINAHL and SPORTDiscus were systematically searched. Twenty-seven papers fulfilled the inclusion criteria and described 67 patients with PCHAP involved in seven sports and three professions. The sports (number of cases) were volleyball (41), baseball (13), swimming (1), tennis (3), American football (2), canoeing (1) and yoga (1) (2 unknown). The professions included a baseball coach, a circus trapeze artist and a mechanic. Apart from expert opinions, no studies have identified risk factors for PCHAP. An anatomic variation of the PCHA origin is suggested to be protective for PCHAP. PCHAP is reported in seven overhead sports and three professions. No risk factors for PCHAP were identified. An anatomic variant of the PCHA origin was found to be a potential protective factor for PCHAP. Physicians should be cautious for distal embolization as a result of PCHAP in athletes and workers, both adult and adolescent, involved in repetitive powerful overhead movements.