Obsessive-compulsive disorder (OCD) is a common neuropsychiatric disorder, with predominant involvement of cortico-striato-thalamo-cortico circuitry. Although late-onset cases (>35 years) usually show an association with various neurological disorders involving basal ganglia and thalamus, it is not the case with the young-onset patients. There have been no reports of OCD comorbid with dysferlinopathy which is usually considered as a disease involving only muscles. However, recently, studies suggest involvement of brain in this disease. Here, we report a case of dysferlinopathy comorbid with OCD and discuss the related literature.