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Epithelial neoplasms of the appendix are rare. They account for an annual age adjusted incidence of 4 cases per 1,000,000. The low incidence of primary appendix tumours means that there are very few published reports of the familial occurrence. This case series reports two cases of primary appendiceal epithelial tumours in two sets of relatives. A genetic evaluation was performed to determine if there was any underlying single gene disorder, which could account for the familial occurrence. The results showed there was no DNA mismatch repair defect evident. This means that the occurrence of appendiceal cancer in siblings may represent a random event. However, exceedingly rare predisposition syndrome cannot be ruled out from our analysis.