A 6Y old boy presented at the Children's Hospital with left adrenal pheochromocytoma, and relapsed with right adrenal pheochromocytoma a year later. His outcome was uneventful in a five year follow-up. His father had left adrenal pheochromocytoma and left extra-adrenal paraganglioma when the father was 11Y, and had an uneventful outcome. A germline VHL missense transversion mutation NM_000551.3:c.488T>G(L163R) was confirmed in both the son and father, and was found by sequencing with polymerase chain reaction (PCR) and analysis with single-strand conformation polymorphism (SSCP). The patient's mother and 3 other siblings had no symptoms and no mutation. Note that the paper describes this variant as c.701T>G since it uses an older reference sequence (Latif et al.).