Pheochromocytomas and paragangliomas are rare neuroendocrine tumors developed from chromaffin cells. They are exceptional in children with an atypical symptomatology. We report here a case of a 17-year-old boy who presented with a left retroperitoneal mass discovered on thoracic-abdominal-pelvic computed tomography (CT) scan in the presence of diffuse abdominal pain, more pronounced in the left hypochondrium. The exploration had objectified an exclusive secretion of urinary normetanephrine over 24 hours. Metanephrine and 3 ortho methyldopamine were within normal limits. He had adrenalectomy after controlling his blood pressure with an alpha blocker. Histology had confirmed a pheochromocytoma of nonaggressive potential Pheochromocytoma of the Adrenal gland Scaled Score (PASS) 2. The evolution was favorable with normalization of blood pressure and urinary catecholamines at one week, three months, six months, and one year. He tested negative for hereditary syndromic including Von Hippel-Lindau (VHL), RET genes, subject to the succinate dehydrogenase complex B and D subunit genes (SDHB-D), which have been requested.

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