Background: Klingsor syndrome refers to genital self-mutilation in man following delusional religious beliefs. Later, Schweitzer proposed expanding the term to include all psychotic disorders, drug and alcohol abuse. Apart from the noted hemorrhagic emergency and difficult surgical operation, there is a psychosocial impact, sexual and urinary complications (stenosis, urethral fistula) which make this pathology complex. Aim: The Aim of this study is to describe the clinical case of Klingsor syndrome and evaluate its management in light of the literature. Case Presentation: We report a rare case of genital self-mutilation in a 35-year-old man, non-compliant schizophrenic, received 04 days after the trauma and whose treatment consisted of remodeling of the penis stump and follow-up by psychiatrists. Conclusion: Klingsor syndrome is a rare but serious phenomenon. Its management remains complex, but it must be early and multidisciplinary.