Papillon Lefevre Syndrome ( PLS ) is a very rare genetic syndrome that only less than 500 cases have reported in the world. Patients have a typical cutaneous involvement with hyperkeratosis especially on the soles and palms and early shedding of primary teeth. Internal organs involvement such as liver abscess has been presented as case reports. This is for the first time that a genetically documented PLS with footsteps of mycobacterium tuberculosis in liver and kidney will report.