The $Papillon-Lef`{e}vre$ Syndrome(PLS), a disease with autosomal recessive inheritance, is characterized by diffuse hyperkeratosis of the palms and soles, mostly prepubertal periodontitis and premature loss of primary and permanent dentition. The etiology of the destruction of periodontal tissues has not been completely clarified. In recent years, two main factors are suggested to be responsible for tooth loss ; firstly, the presence of gram negative microorganisms in the periodontal pockets of the patients. The other factor suggested is cellular deficiency in chemotaxic and phagocytic function of neutrophylic granulocytes. Resent data suggestes that mechanical debridement in conjunction with antibiotic therapy may be successful in periodontal management of $Papillon-Lef`{e}vre$ Syndrome, particularly if administered early. In this study, a $Papillon-Lef`{e}vre$ Syndrome patient was studied clinically, radiologically, histopathologically and microbiologically. 5 years female patient with gingival swelling and destruction of periodontal structure on the whole dentition were examined and palmar and plantar hyperkeratosis were can be seen. On microbiological analysis, Actinobacillus actino-mycetemcomitans was performed. Concurrently, the children recieved extraction of maxillary anterior teeth and construction of removable prosthetis. The combination of professional oral hygiene care and antibiotic therapy improved the dermatologic and periodontal condition.