Childhood-onset obsessive-compulsive disorder affects 1%-2% of children and adolescents. While symptoms reported by children and behavioral therapies and pharmacological interventions administered to children are similar to those seen among individuals who develop obsessive compulsive disorder in adulthood, there are several differences with regards to sex ratios, comorbidity patterns, neuroimaging findings. Family and twin studies support the role of genetics in some forms of obsessive compulsive disorder. Prefrontal cortex, cingulate cortex, thalamus, nucleus caudatus, putamen and globus pallidus are the main brain areas affected in children with obsessive compulsive disorder. Selective serotonin reuptake inhibitors (SSRI) are the treatment of choice for pharmacotherapy of obsessive compulsive disorder and exposure and response prevention are the most commonly applied behavioral therapy methods in obsessive compulsive disorder. Despite advances in the treatment of the disorder, obsessive compulsive disorder is still considered as a debilitating chronic disorder.