Hydrops fetalis refers to the excessive accumulation of fluid in the extracellular compartment of the fetus. Cystic hygroma is a congenital malformation of the cervical lymphatic system responsible for an accumulation of lymphatic fluid commonly in the retrocervical region. Cystic hygromas are most often associated with chromosomal abnormalities. When a cystic hygroma is diagnosed in utero, the fetal survival rate is only 2-6%. When hydrops fetalis is present with cystic hygroma, the mortality rate is close to 100%. We present here the case of a 23-year-old primigravida in whom an ultrasound performed at 17 weeks of amenorrhea revealed a posterior cervical cystic hygroma associated with hydrops fetalis, the evolution of which was marked by intrauterine fetal death two weeks later. Keywords: Hydrops fetalis, Cystic hygroma, Chromosomal abnormalities, Prenatal diagnosis.