Acardiac fetus is a rare lethal fetopathy usually encountered in monoz
ygous pregnancies. Ultrasound prenatal diagnosis has enabled an increa
sing number of observations and raised the need for an adequate therap
eutic approach since the spontaneous prognosis for the healthy twin is
unfavorable in half of the cases. An acardiac fetus was identified at
12 weeks gestation in a 36-year-old woman. Growth of the healthy fetu
s was carefully monitored and progressed normally to delivery by cesar
ean section of a 2.900 kg boy at 36 weeks. At delivery, the acardiac f
etus was found to be totally free of any attachment, floating in the r
emaining fluids. Pathology examination showed a 16 g macerated fetus w
ith a cephalic extremity, a ventral pedicle and a syrenomelic caudal e
xtremity. The caryotype was not significative. Acardiac fetus occurs i
n less than 1% of multiple pregnancies and can develop in single pregn
ancies. Twin reversed arterial perfusion has been recognized as necess
ary for development of the perfused fetus. Genetic and immunologic the
ories have been proposed to explain the pathogenesis which remains unk
nown. Clinical management depends on the spontaneous development of th
e acardiac fetus and the deleterious consequences for the healthy twin
resulting from heart failure, anasarca or prematurity. Medical manage
ment with digoxin, or selective extraction by hysterotomy may improve
prognosis but results have been variable. Echoguided umbilical cord li
gation has also been proposed to improve maternal mortality. Therapeut
ic abstention is no longer indicated at prenatal diagnosis of an acard
iac fetus and a healthy twin despite the risks of invasive treatment.