Isotretinoin embryopathy is an association of malformations caused by the teratogenic effect of isotretinoin (Roaccutane®), a drug marketed for the treatment of cystic acne. The risk of malformations after exposure per os to isotretinoin has been evaluated to be around 20%. The affected infants may present hydrocephalus, microcephaly, ear malformations, conotruncal heart defects and limb abnormalities. There is also increased risk of spontaneous abortion and premature delivery. Isotretinoin may also have effects on child behavior. Isotretinoin is available also as a topical gel. After topical application, plasma levels of isotretinoin and its metabolites are below the limits of detection and present no teratogenic risk. As isotretinoin in its oral form has an half-life of up to 50 hours, most of the drug and its metabolites are eliminated within 10 days of the last dose. For safety reasons it is recommended that isotretinoin should be discontinued at least one month prior to attempting pregnancy. In case of inadvertent exposure during the first trimester of pregnancy, the patient should be informed of a significant teratogenic risk, and even if fetal morphology is found to be normal at ultrasonography, behavioral effects cannot be excluded. Termination of pregnancy may then be considered. Use of isotretinoin in its topical form should also be avoided during pregnancy.