Collodion baby is a rare congenital disorder resembling harlequin fetus but is milder in degree. Although it has been reported that harlequin fetus is associated with kidney abnormalities, malignant keratoma, micromelia, polydactyly, thymic atrophy and thyroid aplasia, there are few reports of collodion baby associated with congenital abnormality and/or disease in the literature. A newborn infant admitted with shedding of the skin and diagnosed as collodion baby concomitant with congenital hypothyroidism is presented here. This is the first case of collodion baby associated with congenital hypothyroidism in the literature to our knowledge.