A series of 28 pheochromocytomas from 28 patients from the Freiburg International Pheochromocytoma Registry were used to study somatic point mutation variants and loss of heterozygosity. Whole blood leukocytes and tumor samples underwent direct sequencing of all three exons of the VHL gene, all eight exons of the SDHB gene, all six exons of the SDHC gene, all four exons of the SDHD gene, and exons 10, 11, and 13 of the RET gene. 9 out of 28 samples were from the VHL germline mutation carriers.VHL-associated pheochromocytoma samples were collected from 1 patient (patient 9) with germline missense (c.193T>A) VHL mutation. The patient was a 14 year old male and presented with multiple benign adrenal pheochromocytomas. The patient was found to have LOH in the SDHC gene.