This case was evaluated by an extra-institutional medical geneticist and according to the EMR was concluded as not associated with a hereditary PGL/PCC syndrome; however, considering the high suspicion of a hereditary PGL/PPC and the elevated prevalence of exon 1 deletion in the SDHB gene documented in our study, it is necessary to complement the genetic studies in this patient with MLPA. Here, SDHB is linked to primary peritoneal carcinoma.