Porphyria cutanea tarda is the most common disorder of porphyrin metabolism in Europe and North America. The disorder is characterized by specific cutaneous lesions, associated systemic findings, and excessive accumulation and excretion of uroporphyrin and coproporphyrins. Reports of this condition associated with pregnancy are scarce in the literature. In this review, we present the case of a patient with porphyria cutanea tarda to illustrate the natural progression and complications of the disorder during pregnancy. Based in this report and a review of the published cases, pregnancy may exacerbate the cutaneous lesions of porphyria cutanea tarda during the first trimester. The incidence of diabetes, antinuclear antibodies, and hepatitis B and C are increased among them, making glucose tolerance and antibody testing mandatory. Newborns should be tested for the disorder during the neonatal period. Genetic counseling is advisable as well as teaching avoidance of provocative factors in affected children.