Holoprosencephaly (HPE) refers to a group of disorders caused by incomplete division of the forebrain with awide range of associated phenotypes [Solomon et al., 2010]. It is themost common forebrain anomaly with a prevalence of 1:250 in embryos and 1:10,000 in liveborn infants [Bous et al., 2012]. Fetal development of HPE may be caused by genetic and teratogenic phenomena [Cohen and Shiota, 2002; Solomon et al., 2010], by aneuploidy (e.g., trisomy 13), or by other genetic syndromes with autosomal recessive, autosomal dominant, or X-linked mutations [Solomon et al., 2000; Cohen and Shiota, 2002]. Diabetes mellitus is a teratogenic risk factor for HPE [Solomon et al., 2000; Cohen and Shiota, 2002]. Mutations in 12 genes are known to cause apparently non-syndromic HPE [Solomon et al., 2010; Bous et al., 2012]. Mutations of four genes, SHH, ZIC2, SIX3, and TGIF, account for most non-chromosomal, non-syndromic HPE [Solomon et al., 2010]. This 24-year-old primigravidwomanpresented for prenatal care in her first trimester. Anatomic screening, performed at 18 6/7 weeks, showed the fetus to have hydrocephalus, alobar holoprosencephaly (HPE), mid-face hypoplasia with bilateral cleft lip and palate, a proboscis, a cervical meningocele, and post-axial polydactyly in the fetal feet (Fig. 1). The family history was unremarkable, and no known environmental or teratogenic exposures were identified. Her hemoglobin A1C was 4.8, suggesting a low likelihood of diabetes mellitus. An amniocentesis was performed and amniotic fluid was tested for karyotype, alpha fetoprotein, and single nucleotide polymorphism (SNP) chromosomal microarray (CMA). The karyotype was 46,XY. CMA showed evidence of isodisomy of chromosome 2 (UPD2) as (arr 2p25.3q37.3 (12, 771–243, 783, 384) 2 hmz). No deletions, mosaicism, or supernumerary chromosomes were noted. Ultrasonography was performed again at 21 4/7 weeks at the patient’s request and the above findings were confirmed. The patient was counseled about her options for continuation and termination of the pregnancy and she opted for termination by dilation and evacuation (D&E), which was performed at 22 weeks gestation. Counseling included that a D&E would preclude the