Introduction. Pheochromocytoma is a catecholamine-secreting neuroendocrine tumor that originates in the chromaffin cells of the adrenal medulla or extra-adrenal sites, both derived from the embryonic neural crest. The term "pheochromocytoma" is reserved for tumors involving the adrenal glands, while for extra-adrenal tumors that come from the sympathetic and parasympathetic ganglia and have a noradrenergic phenotype, the term "paraganglioma" is used. Case Report. Pheochromocytoma is a rare tumor in children. We present the case of a 9-year-old female child with heterotopic retroperitoneal localized pheochromocytoma, clinically manifesting hypertension, tachycardia and headache. The diagnosis was based on abdominal ultrasound, Computer tomography and contrast-enhanced Magnetic Resonance Imaging, confirmed by evaluation of free plasma catecholamine metabolites and testing of fractionated urinary metanephrines. This case is of interest due to an atypical position and vascularization of the pheochromocytoma, located retroperitoneally on the right, casuistically described in the literature, which created some diagnostic confusions. Conclusion. The use of the transperitoneal approach, in this case, allowed an excellent exposure and radical removal of the tumor without injuring the kidney and ureter attached to the tumor.