We report a case of congenital onychodysplasia of the index fingers (Iso-Kikuchi syndrome) in a 2-year-old boy who had nail deformities on both index fingers and the left second toe. He had a micronychia of the left index fingernail, malalignment and abnormal lunula of the right index fingernail and micronychia and malalignment of the left second toenail. Congenital onychodysplasia of the index fingers (COIF) is a rare condition characterized by various forms of nail dysplasia commonly involving the index fingers, but not infrequently also the neighbouring fingers such as the middle fingers and thumbs. The five criteria characterizing COIF include the following: (i) congenital occurrence; (ii) unilateral or bilateral index finger involvement; (iii) variability in nail appearance; (iv)possible hereditary involvement; and (v) frequently associated bone abnormalities. The nails of COIF include the full spectrum of nail dysplasia, from an irregular lunula, malalignment, micronychia (hypoplastic and rudimental), polyonychia (split rudimental), and anonychia, specifically affecting the index fingers. Our patient represents various forms of nail dysplasia of the both index fingers and left second toe such as micronychia, malalignment and abnormal lunula. To our knowledge, the association with second toenail dysplasia in COIF has not previously been reported.