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Plzf (promyelocytic leukemia zinc finger protein) influences limb development as a direct repressor of posterior <jats:italic>HoxD<\/jats:italic> genes. However, the <jats:italic>Plzf<\/jats:italic> coding sequence is intact in the <jats:italic>Lx<\/jats:italic> mutants. Using linkage mapping in F2 hybrids, we downsized the segment containing <jats:italic>Lx<\/jats:italic> to 155 kb and sequenced conserved noncoding elements (CNEs) inside. A 2,964\u2010bp deletion in <jats:italic>Plzf<\/jats:italic> intron 2, never detected in control animals, is the only candidate for <jats:italic>Lx<\/jats:italic>. The deletion removes the most deeply conserved CNE in the 155\u2010kb segment, suggesting a regulatory influence on <jats:italic>Plzf<\/jats:italic> expression. Correspondingly, using in situ hybridization and quantitative real\u2010time polymerase chain reaction, we found a decrease of <jats:italic>Plzf<\/jats:italic> expression in <jats:italic>Lx<\/jats:italic>\/<jats:italic>Lx<\/jats:italic> limb buds with concomitant anterior expansion of expression domains of its targets, <jats:italic>Hoxd10<\/jats:italic>\u2013<jats:italic>13<\/jats:italic> genes, in the absence of ectopic Sonic hedgehog expression. Upstream regulation of Plzf in limb buds is currently unknown. We present here the first candidate Plzf cis\u2010regulatory sequence. 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