The first case of ataxia-telangiectasia (AT) with dysgerminoma of the right ovary, papillary carcinoma of the thyroid, and adenocarcinoma of the pancreas is reported. There is the characteristic trend of the occurrence of the malignant neoplasms in AT. Lymphoreticular neoplasms and leukemia are most frequently seen in the younger patients with AT. On the other hand, epithelial malignant neoplasms are frequently seen in the longer survivors with AT. Literature also shows that nucleomegaly is due to precocious or abnormal aging.