In Norway, the most common porphyrias are acute intermittent porphyria and porphyria cutanea tarda. We describe the first case of porphyria variegata in this country. The patient showed symptoms of the skin and had no abdominal discomfort. Her family was also evaluated and two other persons were found with suspected latent porphyria variegata. We discuss the main symptoms of this type of porphyria and the possibilities of treatment. We emphasize that examination of porphyrins in the feces of patients with porphyria variegata is essential for the diagnosis.