To identify cases of hemivertebra with abnormal cytogenetic studies and assess for other associated anomalies in order to provide more accurate patient counseling and prenatal management. This systematic review was conducted using PubMed (including Cochrane database) and Ovid Medline from inception through November 2019. Studies were deemed eligible for inclusion if hemivertebra with abnormal cytogenetic studies was identified in the fetal, neonatal, or infant period. Studies that described other vertebral anomalies were excluded. We identified 17 cases of hemivertebra with cytogenetic abnormalities among a total of 324 cases (5%) in our review. These include partial tetrasomy 4q, mosaic trisomy 4, mosaic trisomy 7, mosaic trisomy 9, mosaic trisomy 18q, mosaic trisomy 18; trisomy 7, trisomy 15q with monosomy 6q, partial trisomy 22; duplication of 2p; 4p- deletion, 17p deletion, 18p deletion, 18q22.2 deletion, 22q13.3 deletion; ring chromosome 21, and Fanconi’s anemia. 29% (5/17) were diagnosed prenatally, 47% (8/17) at birth, and 18% (3/17) postnatally. All cases had prenatally or postnatally diagnosed associated anomalies. 29% (5/17) had associated anomalies identified prenatally, including ear anomalies, CNS abnormalities, micrognathia, congenital heart defects, renal anomalies, clubfeet, and fetal growth restriction. Postnatally diagnosed anomalies commonly included: CNS (41%, 7/17), cardiac (53%, 9/17), skeletal (71%, 12/17) and genitourinary (59%,10/17). Hemivertebra with abnormal cytogenetic testing was always associated with malformations from other organ systems that could be missed by prenatal ultrasound alone. Those include CNS, cardiovascular, musculoskeletal, and genitourinary anomalies. Fetal echocardiogram is recommended due to the considerable association with congenital heart defects. Fetal neurosonography or MRI should be considered. Of those receiving genetic testing, karyotype or SNP microarray should be considered due to the common presence of mosaicism.