Pathological nighttime fears in children have been little studied. However, this disorder is commonly encountered in medical consultations and is discomforting and dysfunctional for both the child and the family. Most nighttime fears are part and parcel of normal development, and emanate from increasingly sophisticated cognitive development in the growing child. Thus, most children report a variety of coping strategies generally helpful in reducing their anxiety, which resolves spontaneously in the growing child. Nevertheless, in about 10% of children, nighttime fears are related to one or more anxiety disorders according to Diagnostic and Statistical Manual of Mental Disorders criteria. Then, it is estimated that severe nighttime fears and sleep problems occur in 20-30% of children. This problem is not transient and has to be treated. This study aims to review clinical features of nighttime fears and possible treatments for these patients and their families. This systematic review follows the preferred reporting items for systematic reviews and meta-analysis (PRISMA) statement guidelines. Two databases (Medline and Web of Science) were searched combining the search terms: nighttime fears AND children. English and French languages were imposed. There were no publication date or publication status limitations. Pathological nighttime fears are responsible for emotional (crying, panic, tantrums at bedtime, loss of confidence, self-disparaging negative statements, and feeling of social embarrassment) and behavioral (wandering alone in the house at night, calls for parental or sibling comfort, bed sharing with parents or siblings, light source at night, refusal to go to the toilet alone at night) disturbances. This leads to a poor quality of sleep interfering with school learning, and also affects social development and family functioning. A full assessment has to be made to eliminate organic causes, have a baseline functioning, and search for comorbid anxiety diseases. The treatments which have proved effective are some cognitive-behavioral techniques: systematic desensitization (with relaxation or emotive imagery), reinforcement (gain of points and techniques of self statement), and cognitive techniques (reinforcing self-statements, reducing the aversive aspects of being in the dark, involving reality-testing statements, and active control are preferred in children older than 6 years, whereas the "anti-monster letter" and the techniques using a doll are preferred in children under 6 years old). The modelling technique seems to be appropriate at any age. We have explained the clinical features of pathological nighttime fears and the way to assess this disease, and we have pointed out the treatments whose effectiveness has been evaluated in this indication.