Pharmacotherapy and cognitive-behavioural therapy (CBT) have been studied in many of the obsessive-compulsive and related disorders (OCRDs). Serotonin reuptake inhibitors (SRIs) and cognitive-behavioural therapies (CBT) are first-line considerations in many, but not all, of these conditions. There are fewer data available on the combination of these treatment modalities in OCRDs. In obsessive-compulsive disorder (OCD), the SRIs and CBT -which include exposure and response prevention (ERP) -are wellestablished safe and efficacious first-line treatments in adult and paediatric populations. While various pharmacotherapy augmentation strategies have been studied, the most evidence-based approach to date is augmentation with antipsychotic agents. There is also evidence of the value of CBT in the management of treatment-refractory patients. A number of SRIs, such as clomipramine and fluoxetine, have shown efficacy in randomized controlled trials of the pharmacotherapy of body dysmorphic disorder (BDD). There are relatively few data on pharmacotherapy augmentation approaches in BDD. CBT has also been found efficacious in a number of psychotherapy trials of BDD. Less is known about the optimal treatment of the other OCRDs, i.e., hoarding disorder (HD), trichotillomania (hair-pulling disorder, or HPD), and excoriation (skin-picking) disorder (SPD). While patients with HD may have been included in RCTs on OCD, no data from randomized controlled trials of pharmacotherapy specifically for HD have been reported. There is some support for the value of CBT in HD. In HPD, controlled trials of olanzapine, N-acetyl cysteine (NAC), and clomipramine (vs. desipramine) have provided evidence of efficacy. There also is evidence supporting the efficacy of behaviour therapy in reducing hair-pulling. Results from randomized controlled trials of SRIs in SPD have been mixed, with some agents such as fluoxetine and citalopram demonstrating improvement on certain measures of picking behaviour. Behaviour therapy also appears to be useful for SPD.