Endocrine manifestations of neurofibromatosis have been reported in five children, and the literature has been reviewed. Children often show disorders of sexual development and growth, while pheochromocytoma is the commonest endocrine association in adults. Precocious sex development was noted in three children. Two other girls presented with marked retardation of growth. One of them had retarded sex development in association with anorexia nervosa. It has been emphasized that such manifestations may frequently be the presenting features of neurofibromatosis in children.