Neurofibromatosis is an autosomal dominantly inherited neoplastic disorder that is currently incurable and has no obvious contraindications. Neurofibromas are one of the most common and characteristic symptoms of neurofibromatosis. Based on the causative genetic changes as well as clinical manifestations, neurofibromatosis can be broadly classified into neurofibromatosis type I (NF1), neurofibromatosis type II (NF2), and neurocysticercosis (SN). Currently, NF1 accounts for more than 88% of neurofibromatosis, which is a chronic syndrome involving multiple systems, including skin, bone, and nerves, and often develops in childhood and progresses with age, gradually causing pain, deformity, disfigurement, disability, dysfunction, cognitive impairment, and other symptoms. Although there is no cure, early and standardized treatment can slow the progression of the disease and enhance the patient’s mental health. Since the disease is an autosomal dominant disorder, there is no cure. Therefore, couples with the disease who wish to have children should first undergo fertility genetic counseling to discuss the genetic risks associated with the disease and to avoid causing psychological and financial burdens to the family, but it should be noted that pregnancy is not an absolute contraindication. However, for pregnant women with the disease, the hormonal changes associated with pregnancy may lead to the emergence of new neurofibromas and the enlargement of existing neurofibromas, and pregnancy may also increase the incidence of other disorders, including hypertension, preeclampsia, placental abruption and other complications. Therefore, overall, women with this disease need to be evaluated by a medical professional and closely monitored when they become pregnant.