Consensus guidelines recommend plasma-derived C1 inhibitor (C1-INH) as first-line treatment in pregnant women with hereditary angioedema (HAE). We conducted a systematic review of the literature that describes experience with plasma-derived C1-INH during pregnancy. A literature search of PubMed was conducted in November 2018 using variants of “hereditary angioedema” and “pregnancy.” English language articles that presented original data about the use of plasma-derived C1-INH during pregnancy were selected for data extraction. The search returned 253 unique records, of which 40 described the use of C1-INH during pregnancy (91 patients, 136 pregnancies). When the number of doses was reported, a total of 1562 doses were administered ranging from 500 to 3000 IU. Infusions were administered during all 3 trimesters and were most commonly administered during the third trimester. Overall, 1,490,500 IU of plasma-derived C1-INH were administered during pregnancy. Of the 128 fetuses for which outcomes were reported, 3 (2%) resulted in spontaneous abortion, 1 (1%) was stillborn, and 1 (1%) was a vanishing twin. Use of plasma-derived C1-INH in women with HAE during pregnancy has been widely reported in the scientific literature and has a favorable safety profile, supporting treatment guideline recommendations. Copyright © 2020. Published by Elsevier Inc.