2084 In addition to arterial hypertension, the associated diffuse segmental narrowing of the aorta and its branches indicated the association of middle aortic syndrome in this patient, which is a clinical condition generated by segmental narrowing of the descending thoracic and abdominal aorta and their branches, which could be acquired or congenital owing to a developmental anomaly in the fusion and maturation of the paired embryonic dorsal aorta [12]. Conclusion Careful screening of renovascular arterial hypertension is strongly recommended in individuals with neuro-ectodermic diseases, especially epidermal nevus syndrome. Open Access This article is distributed under the terms of the Creative Commons Attribution Noncommercial License which per- mits any noncommercial use, distribution, and reproduction in any medium, provided the original author(s) and source are credited. References 1. Solomon LM, Fretzin DF, Dewald RL (1968) The epidermal nevus syndrome. Arch Dermatol 97:273–285 Pediatr Nephrol (2011) 26:2081–2084 2. Grebe TA, Rimsza ME, Richter SF, Hansen RC, Hoyme HE (1993) Further delineation of the epidermal nevus syndrome: two cases with new findings and literature review. Am J Med Genet 47:24–30 3. Booth TN, Rollins NK (2002) MR imaging of the spine in epidermal nevus syndrome. AJNR Am J Neuroradiol 23:1607– 1610 4. Rogers M, Mccrossin I, Commens C (1989) Epidermal nevi and the epidermal nevus syndrome: a review of 131 cases. J Am Acad Dermatol 20:476–488 5. Aizawa K, Nakamura T, Ohyama Y, Saito Y, Hoshino J, Kanda T, Sumino H, Nagai R (2000) Renal artery stenosis associated with epidermal nevus syndrome. Nephron 84:67–70 6. Kara B, Inan N, Bayramgurler D, Altintas O, Akbulut A (2008) Epidermal nevus syndrome with azygos anterior cerebral artery. Pediatr Neurol 39:283–285 7. Stavrianeas GN, Kakepis ME (2004) Epidermal nevus syndrome. Orphanet Encyclopedia April 2004, http://www.orpha.net/consor/ cgi-bin/OC_Exp.php?Lng=GB&Expert=35125 8. Dobyns WB, Garg BP (1991) Vascular abnormalities in epidermal nevus syndrome. Neurology 41:276–278 9. Fossali E, Signorini E, Intermite RC, Casalini E, Lovaria A, Maninetti MM, Rossi LN (2000) Renovascular disease and hypertension in children with neurofibromatosis. Pediatr Nephrol 14:806–810 10. Dworkin LD, Cooper CJ (2009) Renal-artery stenosis. N Eng J Med 361:1972–1978 11. Booth C, Preston C, Clark G, Reidy J (2002) Management of renal vascular disease in neurofibromatosis type 1 and the role of percutaneous transluminal angioplasty. Nephrol Dial Transplant 17:1235–1240 12. Delis KT, Gloviczki P (2005) Middle aortic syndrome: from presentation to contemporary open surgical and endovascular treatment. Perspect Vasc Surg Endovasc Ther 17:187–203