Acute intermittent porphyria (AIP) is inherited in an autosomal dominant fashion. Only 10% to 15% of the gene carriers have the clinical syndrome. The prevalence of AIP in Europe is 1/20,000. Pregnancy represents an essential risk factor in patients suffering from AIP. The clinical syndrome in AIP presents mainly with acute attacks, especially during the first trimester. Misdiagnosis of AIP unfortunately is very common. Pregnancy in women with AIP is associated with higher rates of spontaneous abortion, hypertension, low birth weight infants and considerable mortality (2-42%). Pregnancy, despite the major hormonal alterations it causes, is seldom associated with porphyric symptoms. There are only limited reports supporting the use of hemin during pregnancy, but experience indicates that it can be safely administered in pregnant women. Until clinical improvement is achieved, symptomatic treatment is recommended. Despite the fact that pregnancy in women suffering from AIP is related to higher rates of morbidity and complications, close management throughout the pregnancy could ensure a good outcome.