Congenital absence of the pericardium (CAP) is a rare condition. Failure to recognize the clinical features of this condition can lead to incorrect and delayed diagnosis. Limited data are available regarding the optimal approach to diagnose and manage patients with suspected CAP. Due to the rare nature of CAP, this condition can present diagnostic and management dilemmas for clinicians. Using 3 cases of CAP as a framework, a clinically focused review on the diagnosis and management of CAP is presented. Clinicians will be provided with a systematic approach to evaluating patients with suspected CAP, incorporating key history, examination, and multimodality cardiovascular imaging investigations.