There are currently no clear guidelines for high-risk breast cancer screening during the pregnancy and breastfeeding periods. The objective of this systematic review (SR) was to assess the available evidence pertaining to breast cancer screening recommendations in this population with the aim of supporting future guidelines. We performed a SR of the literature using the electronic databases MEDLINE and Embase. Predetermined inclusion and exclusion criteria were used during the abstract screening and full-text data extraction phases. We retrieved 2,274 abstracts after removal of duplicates, from which 16 studies were included based on predetermined eligibility criteria. Most of the studies found were narrative reviews and expert opinions. Clinical breast exam (CBE) was recommended by 12 studies during pregnancy and by 6 studies in the breastfeeding period. Mammography was recommended in the breastfeeding period by 2 studies. Magnetic resonance imaging was recommended in the breastfeeding period by 2 studies. Ultrasound was considered not appropriate for screening in this population. The information extracted from this SR is based primarily on expert opinion and anecdotal evidence, which explains the lack of standardized guidelines for high-risk breast cancer screening in this population. However, expert opinion may be a surrogate outcome for high-risk breast cancer screening recommendations in this subset of patients, and as such, may justify the clinical management to be tailored accordingly. This SR summarizes the evidence pertaining to high-risk breast cancer screening during pregnancy and breastfeeding, which could serve as a catalyst for future research on the topic.