Narcolepsy with cataplexy is a rare but long lasting and disabling disorder where onset often occurs during childhood. Excessive daytime sleepiness, irresistible sleep attacks, partial or complete cataplexies leading to abrupt falls without loss of consciousness are the major symptoms of the disorder together with hypnagogic hallucinations and sleep paralysis. Narcolepsy cases with childhood onset are often severe. School and social life are affected in children suffering from this condition. Due to the permanence of the disorder, long-term treatment is often required targeting sleepiness. Anticataplectic treatments may be necessary early on in the course of the disease, when considering the potential handicap created by the cataplexies and the risk of falls. There is no medication available to this day to cure the disorder. Familial education, psychological and academic support play a crucial role in the management of the symptoms and in, combination with pharmacological treatment, the quality of life in children with narcolepsy and their outcome in adulthood should lead to an improvement.