Homeobox genes are an evolutionarily conserved class of transcription factors that are key regulators during developmental processes such as regional specification, patterning, and differentiation. In this review, we summarize the expression pattern, loss‐ and/or gain‐of‐function mouse models, and naturally occurring mouse and human mutations of known homeobox genes required for the development of ectodermal appendages. J. Cell. Physiol. 216: 337–346, 2008. © 2008 Wiley‐Liss, Inc.