Tehran University of Medical Sciences Acta Medica Iranica 0044-6025 52 8 2014 08 15 647 649 EN Azar Nickavar Department of Pediatric Nephrology, Aliasghar Children's Hospital, Iran University of Medical Sciences, Tehran, Iran. Najmessadat Atefi Department of Dermatology, Aliasghar Children's Hospital, Iran University of Medical Sciences, Tehran, Iran. Kambiz Kamyab Hesari Department of Pathology, Razi Dermatology Hospital, Tehran University of Medical Sciences, Tehran, Iran. 2015 10 11 Epidermal nevus syndrome is a rare congenital disorder, characterized by epidermal nevi and multiple organ involvement. Multicystic kidney disease has been very rarely reported in this syndrome. Here is the report of a boy presented with multiple epidermal nevi, cardiac anomaly, seizure attack, hemi hypertrophy, and multicystic dysplastic kidney complicated with Wilms' tumor. According to this association, it is suggested to search for dysplastic kidney disease in patients with neurocutaneous disorders. https://acta.tums.ac.ir/index.php/acta/article/view/4558 https://acta.tums.ac.ir/index.php/acta/article/download/4558/4353