The records of 110 patients (58 females and 52 males) with histologically proven adrenal cortical carcinoma were studied. Fifty-six of 110 (50.9%) patients had abdominal symptoms and 33 (30.0%) had a palpable abdominal mass. Surgery for localized and regional disease was associated with a disease-free survival time of at least 2 years in 28 of the 50 (56%) patients. Abdominal radiotherapy, systemic chemotherapy, and 1,1 dichloro-2 (O-chlorophenyl)-2 (P-chlorophenyl) ethane (OPDDD) were effective in three of 19 (15.8%) patients, three of 31 (9.7%) patients, and 21 of 72 (29.2%) patients, respectively. Combined radiotherapy and OPDDD did not confer any additional benefit over OPDDD alone. Six of seven patients who received OPDDD as adjuvant treatment are still alive at 1 to 4 years. Distant metastases occurred most commonly in the lungs, liver, peritoneum, lymph nodes, and bones. Only 23% of the patients survived 5 years or more. We conclude that early diagnosis and complete surgical excision offer the best prospects for long-term survival and that the efficacy of adjuvant OPDDD needs to be evaluated further.