Extramammary Paget disease (EMPD) is a rare skin tumor that manifests as poorly delimited lesions located mainly in genital area. Prognosis correlates with thickness and dermal invasion. The gold standard for diagnosis is the histopathological study of a biopsy. However, this technique is invasive and only shows a small area of the tumor. A good correlation has been reported between ultrasounds (US) and histopathologic finding. Moreover, a US examination has the advantages of wide availability, noninvasiveness, low cost, ease of use, and real‐time scanning. We review the ultrasound signs described for this disease.