Koro syndrome has been colorfully described as a pathological distortion of one’s body image of the genital organ. In Koro, body image dysphoria is characterized by severe anxiety related to the delusional idea that one’s genitals will shrink and retract into one’s abdomen, eventually leading to death. This syndrome was first reported in South East Asia, where endemics have been described, but it has also sporadically occurred globally. We present a systematic literature review on Koro syndrome and report 7 cases from Canada. A search review with PubMed and Google Scholar resulted in 504 entries. Sixty-seven manuscripts were eventually selected following a thorough elimination process. The resultant literature underscored the cultural diversity that underlay the reported cases. Various aspects of Koro have been examined (eg, etiological, clinical, diagnostic, and cultural aspects). It has stimulated substantial scholarly debate, discussions, correspondences, and arguments from anthropological, psychiatric, psychological, and biological perspectives. In our series, it seems that Koro could have been misattributed here. The primary concern was not with penile retraction of the cases. To our knowledge, this is the first time that a series of cases is documented from North America where the syndrome is often ignored. We highlight the potential differences between the classical Koro syndrome and a collection of beliefs related to the perception or delusion of penile retraction in other codable psychiatric disorders, Koro-like syndrome. Understanding Koro syndrome beyond geographic boundaries is in line with our collected case reports of Koro from outside Asia.