Massive fetomaternal haemorrhage (FMH) occurs in 0.12 to 0.5% of pregnancies. It is most often spontaneous and involves uncomplicated near-term pregnancies. It causes fetal anaemia, with or without fetal distress and hydrops fetalis. To our knowledge only one paper has reported a neurological complication (hemiplegia). We describe one case of FMH (maximal Kleihauer test = 6.5%) at 28 weeks gestation, which was spontaneous, reversible, associated with sinusoidal fetal heart rare (FHR) and hydrops fetalis; and complicated by an intraventricular antenatal haemorrhage at 30 weeks gestation. Echographic abnormalities decreased. The infant was born at 40 weeks gestation. Clinical examination was normal during the first week of life. At the age of 4 1/2 months, examination showed axial hypotonia and moderated dilatation of intracerebral lateral ventricules without any other brain damage. At the age of 24 months, the child had retarded walking and hypotonia. The outcome was spontaneously favourable with disappearance in utero of the intraventricular haemorrhage (HIV), without hydrocephalia or ischaemic lesions. Three cases of similar FMH have been reported but none of them described cerebral complications. Intrauterine intravascular transfusion should be proposed early. No single pathophysiological mechanism of FMH has been universally accepted and there is no aetiological treatment. The risk of recurrence of FMH in later pregnancies requires careful follow-up.