Tabriz University of Medical Sciences Medical Journal of Tabriz University of Medical Sciences 2783-2031 35 2 2013 06 05 ARC Syndrome; Arthrogryposis, Renal and Cholestasis 106 109 EN Majid Malaki Armen Malekian Mandana Rafeey Anoush Azarafar Journal Article 2012 08 15 2012 09 24 ARC syndrome is an abbreviation of arthrogryposis, renal and cholestasis. It is a rare syndrome but needs awareness of physicians. During past 4 years two cases with direct hyperbilirubinemia, polyuria, ichtiosis and skeletal malformation have been diagnosed, both of them were term born from consanguine parents, and present their symptom and signs during the first month of birth. Their liver transaminase was mildly evaluated and one of them had proximal renal tubular acidosis and another one had combined proximal and distal renal tubular acidosis. ARC presents itself by prolonged neonatal icter, unilateral or bilateral arthrogryposis, and variable renal findings but polyuria, polydypsia and acidosis are constant renal findings of ARC.