RESUMO
Acute intermittent porphyria [AlP] is a rare n disorder of heme biosynthesis characterized by enzymatic defect of porphobiligen desaminase with accumulation and s excretion of porphyrins and their precursors. Clinical picture is characterized by attacks with a triad of abdominal pain psychiatric disorder and neurological involvement [central and Peripheral nervous system manifestations, often precipitated porphyrinogenic medications are of poor outcome. We report a new cases A 13-year-old girl who: several attacks of AlP and developed acute severe axonal motor neuropathy, three weeks after porphyrinogenic [Famotidin, Phenobarbital and Nifedipine]. We stress on the importance of early diagnosis of AIP to prevent serious neurological complications often precipitated by medications and the efficiency of heme arginate treatment when administrated early during the attacks