Dandy-Walker variant (DWV) might be considered as one of the borderlines in the examination of fetal brain. We report the first case of a DWV in a fetus conceived by ICSI. A 34 year-old woman underwent assisted reproductive treatment due to male factor infertility. A twin gestation was achieved, one of the fetuses was diagnosed as DWV on 22 weeks’ ultrasound examination. Fetal MRI confirmed the diagnosis. The couple did not allow fetal karyotyping. Pregnancy continued uneventfully. Associated cardiac and minor skeletal anomalies were seen in the postpartum examination of the affected newborn. She died six days after birth due to pulmonary insufficiency. Karyotype analysis of the affected infant performed after birth was reported to be partial trisomy 9. Parental karyotype analysis was further performed and balanced reciprocal translocation t(4;9) was found in the mother. A careful posterior fossa evaluation should be performed as part of routine anatomical survey in ICSI conceptions. Upon the detection of an anomaly, a comprehensive fetal ultrasound for associated abnormalities and karyotype analysis are vital for obstetric management and neonatal survival.