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Postpartum cerebral angiopathy (PCA) is a rare and pathophysiologically ill-characterized cerebral vasoconstriction syndrome, occurring within 30 days of a usually uncomplicated pregnancy and delivery. Its onset has been associated with the use of vasoactive medications, particularly ergot alkaloids. Other cases have occurred in the absence of these medications, prompting conjecture into possible overlap between PCA and other conditions known to cause cerebral vasoconstriction, including primary angiitis of the central nervous system and postpartum eclampsia. The vast majority of cases follow a relatively benign course; however, a fatal case has been reported. Histopathologic findings in PCA, so far limited to the fatal case and two more recent biopsies, have been nonspecific.Here we present a second fatal case of PCA, including pre- and post-mortem histopathologic analysis. We also include a review of all PCA cases reported in the English literature.Criteria for the clinical diagnosis of PCA are proposed and used to select case reports from the medical literature. Data pertaining to patient characteristics, clinical symptomatology, cerebral imaging findings, and clinical outcomes are compared between cases associated with the postpartum use of vasoactive medications and spontaneous cases.We conclude that histopathologic findings in PCA are nonspecific and secondary to ischemic brain injury. Functional vasoconstriction is the most likely primary pathophysiologic process in PCA. The etiology in cases associated with medications may be due to idiosyncratic reactions to these agents. Significant overlap in symtomatology and clinical features exists between spontaneous cases and late postpartum eclampsia.