Most familial pancreatic carcinomas have been reported from European countries and the United States, and there has been only one report from Japan. A 50-year-old Japanese woman presented with a pancreatic head mass and underwent pylorus-preserving pancreatoduodenectomy with portal vein resection. The histological diagnosis was well-differentiated adenocarcinoma of the head of the pancreas. Her mother died of pancreatic head carcinoma, which had been shown on computed tomography at the age of 70 years. One of her uncles on her father's side had had pancreatic tail carcinoma, and at the age of 59, had undergone distal pancreatectomy, splenectomy, wedge resection of the liver, and partial resection of the colon. The histological diagnosis was moderately differentiated tubular adenocarcinoma of the pancreas. He had had a subtotal gastrectomy for early gastric cancer (tubular adenocarcinoma limited to the mucosa) at the age of 53. He died of recurrence of the pancreatic tail carcinoma 3 months after the distal pancreatectomy had been performed. This communication reports a second Japanese family with familial pancreatic cancer, as shown by pancreatic carcinomas in two first-degree relatives and in one third-degree relative.