European Journal of Dentistry and Medicine1996-3378xxxx-xxxxAcademic Journals Inc.10.3923/ejdm.2012.1.7M. XavierArun RaiKavita M. HegdeAmitha JoshiSaurabh 1201241Oral-facial-digital syndrome type 1 (OFD1) is characterized by an X-linked dominant mode of inheritance with lethality in males. It presents with peculiar malformations in the oral cavity and defects in the face and digits of the upper and lower extremities. Accurate diagnosis following clinical investigations by allied health professionals is indispensable to plan out a systematic management protocol in these victims in order to minimize future odontogenic problems. This report presented a unique case of females in a family suffering from manifestations of OFD1 syndrome since three generations. The characteristic clinical features of all the female members were promptly identified, investigated and the inter-disciplinary management protocol that was systematically instituted is depicted in this report.]]>Al-Qattan, M.M.,199851476479Coll, E., R. Torra, J. Pascual, A. Botey and J. Ara et al.,19971210401042Del, C.B.M., N. Primc, H. Veliche, S. Rosales and R. Carrero-Valenzuela et al.,199916367370Ferrante, M.I., G. Giorgio, S.A. Feather, A. Bulfone and V. Wright et al.,200168569576Gorlin, R.J., V.E Anderson and C.R. Scott,1961264486489Gorlin, R.J. and J. Psaume,196261520530King, N.M. and A.M. Sanares,200226211215Macca, M. and B. Franco,2009151318325Mihci, E., S. Tacoy, G. Ozbilim and B. Franco,200744854856Sousa, Y.T.S. and D.D. Kanaan,1994577174Razafindrabe, A.B., V.H. Randriamanantenasoa, M.F. Andrianasolo, J.B. Radaviarison, V.O. Rasoarimasy and J.D. Rakotovao,2007711081111Kaya, S., N. Hamamci, I. Yavuz, O. Adiguzel and E.C. Tumen,200832430Ogundele, B.O. and S.E. Ogunsile,200816471Mohammadian, S. and H. Khoddam,2007712061209Madukwe, I.U.,20077701703Motlagh, M.G., G.R.J. Khaniki and H. Adiban,20077116120Mahvi, A.H., M.A. Zazoli, M. Younecian, B. Nicpour and A. Babapour,20066658661Zeraati, H. and M.G. Motlagh,2006614Malekipour, M.R., M. Messripour and F. Shirani,20085280283