When the histological features characteristic of MLA, including compactly aggregated small tubules, eosinophilic dense intraluminal secretions, architectural diversity, and high nuclear-to-cytoplasmic ratio, are observed, it is recommended to perform immunostaining for hormonal receptors and markers of mesonephric lineage to correctly determine the histological type. Here, NR4A1 is linked to microphthalmia with limb anomalies.