Pediatr Nephrol (2011) 26:2081–2084 DOI 10.1007/s00467-011-1949-z BRIEF REPORT Bilateral renal artery stenosis and epidermal nevus syndrome in a child Fahad Alsohim & Pauline Abou-Jaoude & Jean Ninet & Jean-Pierre Pracros & Alice Phan & Pierre Cochat Received: 14 December 2010 / Revised: 25 May 2011 / Accepted: 31 May 2011 / Published online: 1 July 2011 # The Author(s) 2011. This article is published with open access at Springerlink.com Abstract Epidermal nevus syndrome is a rare congenital sporadic neuro-ectodermic disorder, characterized by the presence of epidermal nevi in association with various developmental abnormalities of the skin, eyes, nervous, skeletal, cardiovascular and urogenital systems. We describe a 5-year-old boy with conjunctival lipodermoid, cervical and facial sebaceous nevi who presented at 3 years of age with hypertension due to bilateral renal artery stenosis together with multiple vascular anomalies (aorta, celiac trunk, superior mesenteric artery) as shown by magnetic resonance angiog- raphy. Systemic arterial hypertension was difficult to control despite combined anti-hypertensive drugs and the surgical repair of the aortic coarctation. F. Alsohim (*) : P. Abou-Jaoude : A. Phan Service de néphrologie, rhumatologie et dermatologie pédiatriques, Hôpital Femme Mère Enfant, centre de référence des maladies rénales rares, Hospices Civils de Lyon, 59 boulevard Pinel, 69500 Bron, France e-mail: alsohim-md@hotmail.com J. Ninet Service de chirurgie cardio-vasculaire, Hôpital Louis Pradel, groupement hospitalier Est, Bron, France J.-P. Pracros Service de radiologie, Hôpital Femme Mère Enfant, Bron, France P. Cochat Université de Lyon, Lyon, France F. Alsohim King Saud university, Riyadh, Kingdom of Saudi Arabia Keywords Epidermal nevus syndrome . Hypertension . Aortic stenosis . Renal artery stenosis Introduction Epidermal nevus syndrome is a group of congenital neuro- ectodermic disorders characterized by epidermal nevi in association with cerebral, ocular, skeletal, and sometimes cardiac and renal abnormalities. In 1968, Solomon et al. [1] reported the first case of epidermal nevus syndrome. While universal diagnostic criteria have not yet been defined, the diagnosis is usually based on epidermal or sebaceous nevus on the scalp, face, trunk or extremities present at birth or appearing in early childhood, accompanied by various anomalies of the central nervous system, bones, and eyes [2]. Vascular anomalies associated with epidermal nevus syndrome are rare find- ings, including aneurysm, arteriovenous malformation, vascular coarctation, patent ductus arteriosus, and vascular dysplasia [3, 4]. Only one case of arterial hypertension secondary to renal artery stenosis and narrowing of abdominal aorta in association with epidermal nevus syndrome has been described so far [5]. We report on a 5- year-old boy with epidermal nevus syndrome who pre- sented with arterial hypertension due to bilateral renal artery stenosis in association with other severe vascular malformations. Case report A 5-year-old boy with epidermal nevus syndrome without neurological abnormality, diagnosed at birth because of right hemifacial and cervical nevi together with conjuncti-