Wolfram syndrome (WS) is a condition characterized by diabetes insipidus (DI), diabetes mellitus (DM), optic atrophy and deafness. DM symptoms appear first, than optic atrophy develops in the first decade, and DI and sensorineural deafness occur in the second decade. Etiopathology of the syndrome is not clear, however, inheritance of the syndrome is autosomal recessive. Generally, mutations of WFS1, placed on the 4th chromosome, are responsible of the syndrome. Main diagnostic criteria of the syndrome are juvenile DM and optic atrophy. Atony of urinary tract, ataxia, hypoşexia, mental retardation and psychiatric disorders are other defined clinical features. Although the co-existence of other type of psychopathology was documented in the literature, there is no report of co-existence of WS and Obsessive Compulsive Disorder (OCD). Here we report a child with WS with co-existent OCD, whose OCD symptoms responded şuoxetine plus aripiprazole treatment. Case Report: A ten years old female is referred by pediatric endocrinology department with complaints of obsessions, irritability, peer problems and aggression. Previously, the child was diagnosed as DM, after applying the hospital with recurrent urinary tract infections. She also had bilateral optic atrophy diagnosis. Genetic analysis was performed and a mutation on WFS1 gene was identified. After psychiatric assessment, we made diagnosis of OCD according to DSM-IV and started medical treatment with şuoxetine 10 mg/day and gradually increased to 30 mg/day. There has been no response to the treatment for 2 months. Hence, we started aripiprazole 5 mg/day. Fluoxetine 30 mg/day and aripiprazole 5 mg/day treatment led to partial remission in 2 months. She is followed up with partial remission with these treatment modalities. In the literature, some psychiatric disorders include aggressive behaviours, increased suicide risk, paranoid delusions, schizophrenia, resistant depression are identified in patients with WS, and genetic studies documented a weak linkage between psychopathology and exon 8. The co-existence of WS and OCD may be meaningful in terms of shared etiological factors. On the other hand co-existent OCD seems to respond usual psychotropic medications.