Scientific BACKGROUND: Fixed dystonia, is an immobile dystonic posture which could not return to neutral position at rest. Clenched fist syndrome, which is an isolated form of fixed dystonia of hands, could be confused with focal hand dystonia. Fixed dystonias could be seen in symptomatic dystonias (such as corticobasal degeneration, acquired basal ganglion disease), complex regional pain syndrome, and psychological movement disorder. The diagnosis of this kind of dystonias may be delayed and the treatment is difficult. OBJECTIVE: Our aim is to present a case with clenched fist syndrome, to discuss the differential diagnosis, treatment and to review of the literature. CASE: The patient is a 42-year-old woman with inability to use her right hand for 5 and left hand for 3 years. In physical examination, dorsum of the hands were oedematous, palms of the hands were macerated with a bad odour, and unguis had a dystrophic appearence. In neurologic examination, clenched fists were observed. Voluntary and forced extension of the interphalangeal and metacarpophalangeal joints were impossible. After general anesthesia, passive extension of the hands were only minimal. Cranial, spinal magnetic resonance imaging and blood chemistry were within normal limits. In needle electromyographic study dystonic discharges were not observed. Multidisciplinary approach was performed in management. CONCLUSION: In clenched fist syndrome or generally in fixed dystonias, invasive treatment modalities had to be avoided. Treatment modalities including physiotherapy, work-therapy, behavioural therapy, psychotherapy, botilinum toxin injection, medical treatment such as anticholinergics, benzodiazepine and antiepileptics should be performed by multidisciplinary approach after primary and secondary etiologies were eliminated. This means neurologist, physiotherapist, psychiatrist, dermatologist, and hand surgeon should work together when dealing such a patient.