A highlighted case for emphasizing on clinical diagnosis for rare syndrome in third world

Premature tooth loss is a disastrous situation may impact on deciduous or permanent teeth era with different cause. It is may be attributed to some disorders like papillon- lefevr syndrome or coffin-lowry syndrome but because of ambiguous nature, precious diagnosis is not easily possible .having very low incidence and defined by few and limited case series ,with vague characters to some extent, confusion in detecting the right diagnosis is a common possibility. Then it's expectable to have a wrong diagnosis for this case. In this article, a 5 year old boy with chief complaint of early tooth loss despite having blindness in left eye and palmar keratosis is reported, although he had some other manifestation of ODDD like ataxia, dysartheria and nail deformity, ignoring other extra and intra oral finding, he was diagnosed as papillon- lefevr syndrome before, just because of early tooth loss and palmar keratosis.