This is the first report in the English literature of the birth of an obligate SS infant whose parents both had sickle cell anemia. The young adult SS parents of this infant and their families did not wish to prevent the birth of this child but had, in fact, many positive reasons to continue the pregnancy to term even in the face of the increased personal risk of pregnancy for this mother. This unusual circumstance initiated a re-appraisal of the realistic expectations with respect to reproduction in patients with homozygous recessive genetic disorders. No definitive studies could be found wllich attempted to evaluate the influence of genetic counseling on reproductive behavior in patients with sickle cell anemia. The questions raised abut the‘right to reproduce’and the realistic aims of genetic counseling in adult homozygote patients have been discussed. However, no conclusions seem warranted because of the paucity of available information.