Obsessive-compulsive disorder (OCD) is characterized by cognitive regulation deficits. However, the current literature has focused on executive functioning and emotional response impairments in this disorder. Herein, we conducted a systematic review of studies assessing the behavioral, physiological, and neurobiological alterations in cognitive regulation in obsessive-compulsive patients using the PubMed database. Most of the studies included explored behavioral (distress, arousal, and frequency of intrusive thoughts) and neurobiological measures (brain activity and functional connectivity) using affective cognitive regulation paradigms. Our results pointed to the advantageous use of reappraisal and acceptance strategies in contrast to suppression to reduce distress and frequency of intrusive thoughts. Moreover, we observed alterations in frontoparietal network activity during cognitive regulation. Our conclusions are limited by the inclusion of underpowered studies with treated patients. Nonetheless, our findings support the OCD impairments in cognitive regulation of emotion and might help to improve current guidelines for cognitive therapy.