Introduction:Filariasis is an endemic infection in tropical and subtropical countries. The disease is caused by para-sites from the group filarodidae. Epidermolysis Bullosa, on the other hand is a group of rare genetic skin diseases that characterize by skin blister and erode facilely. Due to rarity of Epidermolysis Bullosa and uncommon occurrence of Filariasis, there is extremely limited case or paper reporting on safety profile of medication that are used to treat Filariasis patient with underlying Epidermolysis Bullosa.Serious adverse event that is anticipated in this cohort of patient are Stevens-Johnson syndrome and Mazotti reaction. Case description: Surveillance activity is necessary in high endemic localities in Sabah in order to control the spread of this mosquitoes-borne disease. The available tool is Brugia RapidTM kit, a test kit that detects filarial antibodies.A 13 year-old boy with underlying Epidemolysis Bullosa Simplex was detected during surveillance activities. It was further confirmed with night blood on microscopic slide that depicted high density of parasite (microfilaria count: 31). The WHO specifically exempted the following groups from the treatment - children under 5 years of age; pregnant women; and seriously ill individuals i.e. those who are having acute or chronic illness that makes them too sick or weak to get out of bed; and those with an illness who are life-dependent on medical intervention. This is because ingestion of the medications can result in adverse events due to the destruction of killed parasites. No guideline is available for treatment of lymphatic filariasis in rare genetic disorders. Conclusion: The recommended dosage for IDA is Ivermectin 3mcg/kg, Diethylcarbamazine 6mg/kg and Albendazole 400mg for positive patient yearly. Patient was admitted in hospital for observation treatment with the suggested dose. From the case study it shows it is safe to treat this cohort patient. However, it is advisable to treat such rare cases by case basis and in comparison to others where treatment is given in the community this patients should be treat in more control environment such in the hospital.