&NA;Thirty‐nine patients (age 40 years and younger) with rectal cancer treated at the Mount Sinai Hospital between 1967 and 1985 were studied. Their mean age was 34 years (range, 21 to 40). A positive family history for colorectal cancer was found in six patients (15 percent). Fifty percent of patients under age 30 had metastatic disease at diagnosis. Twenty‐seven patients (69 percent) had potentially curative resections. Of these, 17 (63 percent) had lymph‐node metastasis. This rate is twice as high as in a group of 315 patients with rectal cancer over age 40 (31 percent). The overall five‐year survival for young patients having curative resection was 53 percent. Noncolorectal cancer occurred in three patients in this series and six patients also had first‐degree relatives with noncolorectal cancer. Young patients with rectal cancer appear to belong to a high‐risk cancer group which often seems to have a genetic pattern of predisposition.