In an attempt to determine the natural history of ductal adenocarcinoma of the pancreas in patients under 40 years of age, we reviewed the surgical outcomes of all such patients seen at the Mayo Clinic from 1970 to 1985. Histologic sections were reviewed; islet cell tumors and cystadenocarcinomas were carefully excluded. Twenty-six patients were identified. Their mean age was 34 years, with only one patient less than 25 years old. Symptoms included primarily abdominal pain, weight loss, and jaundice. One third of patients had a recent or past history of pancreatic disease including pancreatitis, pseudocysts, benign cystadenoma, and choledochal cyst. The tumor was located in the head of the gland in 62% of patients. "Curative" resections were possible in only three patients (12%); the remaining patients underwent palliative bypass (38%), biopsy alone (42%), or a palliative resection (8%). The hospital mortality rate was 12%, with actual 1-, 2-, and 5-year survival rates of 19%, 8%, and 4%, respectively, with a median survival of 4 months. The only long-term survivor underwent biliary bypass at age 15 years for a large neoplasm in the head of the gland; despite biopsy-proved liver metastases at that time, she continues to do well 5 years later. Histologic review indicated this tumor to be a "solid and papillary neoplasm of the pancreas." Ductal adenocarcinoma of the pancreas in young patients is an aggressive tumor with a poor prognosis behaving much like ductal adenocarcinoma in older patients (greater than 40 years). In rare instances a more favorable outcome can be expected when a solid and papillary neoplasm is found.