Less than 1% of Pap smears are interpreted as having atypical glandular cells. Because of the rarity of diagnosis, providers are frequently unfamiliar with both the workup and the potential ramifications. Comprehensive evaluation is required in all cases to exclude a spectrum of possible diagnoses. Although colposcopy, human papillomavirus DNA testing, endocervical curettage, and endometrial sampling should be the initial part of the evaluation, these procedures may not identify any specific etiology. The aim of this review is to provide the most current strategy for management of this rare, but suspicious Pap test result.