EVX1 encodes a protein of 407 amino acids containing a homeodomain closely related to the Drosophila even-skipped (eve) segmentation gene of the pair-rule class. EVX1 belongs to a small family of vertebrate eve-related homeobox genes including human EVX1 and EVX2, their murine homologs Evx-1 and Evx-2, and the frog Xhox-3 gene. The human EVX2 gene is located at the 5-prime end of the HOX4 locus on chromosome 2 (see HOXD13).

Faiella et al. (1991) showed that the EVX1 gene is located at the 5-prime end of the HOX1 locus on chromosome 7, 48 kb upstream from the most 5-prime of the 11 HOX1 genes, namely HOX1J (HOXA13). Both EVX genes are transcribed in an opposite orientation as compared to adjacent HOX genes.