Body Integrity Identity Disorder (BIID), also known as body integrity dysphoria and xenomelia is a rare chronic psychiatric condition characterized by an intense and persistent desire to have one or both healthy limbs amputated or paralyzed. Affected individuals usually experience discomfort with being able-bodied. This disorder often causes significant distress and decreased quality of life, with patients believing they would be happier living as an amputee, sometimes mutilating themselves. Though very rare, most sufferers fail to complain to clinicians due to the embarrassing nature of their bizarre desire. As a result, the disorder is infrequently studied and lacks standardized diagnostic criteria and formal guidelines for psychotherapy or pharmacotherapy, underscoring the need for investigation and extensive empirical research. This study reviews the current knowledge and management of BIID to encourage recognition by the Diagnostic and Statistical Manual of Mental Disorders (DSM) and the International Classification of Disease (ICD).