Ciliated hepatic foregut cysts (CHFC) are rare, typically benign, lesions whose incidence has been increasing recently. Despite this increase in incidence, they remain rare and several key characteristics remain poorly understood, including the range of presentation and the risk of malignant conversion. To better understand CHFC, an extensive review of the international literature was performed. Characteristics including size, location, contents, presenting symptoms, and risk of malignancy were analyzed. In addition, an illustrative case is presented to highlight a potential pitfall in diagnosis: Although the presentation is thought to be typically painless or vaguely painful, these lesions may also present with hemorrhage and sudden severe abdominal pain. Importantly, although malignant degeneration is uncommon, analysis revealed that malignancy is significantly associated with size, which was significantly larger (median 10 cm) in malignant CHFC compared with the typical benign CHFC (3 cm) (p < 0.01). Large or symptomatic cysts, or small asymptomatic cysts that are atypical, should be resected.