Objectives: Carrying out a pregnancy to term in a patient with a paraganglioma or pheochromocytoma (PPGL) results from a multidisciplinary challenge. The objective was to compare our therapeutic attitude with the existing literature and to identify optimal treatment.Methods: Description of a case of paraganglioma treated during pregnancy at the University Hospital Center of Caen (France) in 2024 and comparison with the literature.Results: We describe a patient with a family history of paraganglioma, in whom paravesical PPGL was discovered at 18 weeks of gestation. The symptoms were marked by episodes of palpitations and headaches during urination. The diagnosis was confirmed by a measurement of urinary metanephrines associated with magnetic resonance imaging. After initiation of alpha-blocker treatment, delivery was performed by cesarean section at 36 weeks of gestation. An excision was carried out by laparoscopy 2 months postpartum without intra- and post-operative complications.Conclusions: The absence of antenatal diagnosis of PPGL is the main risk factor increasing maternal and fetal mortality. Certain criteria such as a family history, the appearance of early or malignant arterial hypertension should suggest the diagnosis of PPGL. The initiation of alpha-blocker treatment upon diagnosis is essential. The curative therapy remains surgical excision. Its timing depends on its size, location, term of pregnancy and route of delivery. It is preferable to postpone it until postpartum in order to reduce the risk of complications.