Fertility and reproduction are now frequent questions for cystic fibrosis adult patients. Females are sometimes subfertile because of abnormal cervical mucus viscosity or anovulatory cycles. After genetic counseling and evaluation of respiratory prognosis, assisted reproduction can be proposed. Fertility is frequently normal and contraception should be proposed. Oral contraception appears to be safe. Pregnancy in cystic fibrosis women should be undertaken after genetic counseling and clinical evaluation; the prognosis for the mother is not modified for patients with stable pulmonary status and FEV1 up to 30% of predicted with correct nutritional status. Preterm delivery is frequent. Usual therapeutics should be continued unless ursodeoxycholic acid or nonsteroidal antiinflammatory drugs. Usual antipseudomonas antibiotics can be used; pulmonary exacerbations have to be treated early. Almost all cystic fibrosis men are infertile because of congenital bilateral absence of vas deferens (CBAVD). CBAVD can revealed incomplete cystic fibrosis phenotypes associated with specific genotypes. After genetic counseling, intracytoplasmic injection after epididymal puncture can be proposed. In all cases, it is important to evaluate the life prognosis, before pregnancy or paternity and to propose genetic counseling, essentially depending on the genotype of the spouse.