Wilson's disease (hepatolenticular degeneration) is a rare autosomal recessive disorder that usually occurs between the first and third decades. The condition is characterized by excessive deposition of copper in the liver and brain. It is fatal if the diagnosis and treatment are not performed early in life. The authors describe one case of pregnancy and Wilson's disease, showing the good evolution of the patient, who used D-penicillamine until the seventh week of gestation, discontinuing thereafter until the 20th week of gestation. The mother evolved with no signs of clinic and obstetric problems. The newborn did not present any problem and was healthy in his first year of life.