To evaluate men with severe osteoporosis for pathogenetic factors and to review the reported features of primary osteoporosis in men. Case series and clinical review. 47 men consecutively referred to a metabolic bone center because of atraumatic (or minimally traumatic) fractures (91%) or radiographic osteopenia (9%). Clinical assessment, radiographs, chemical analyses of serum and urine, hormone assays, skeletal densitometry, and histomorphometry of iliac crest biopsy specimens. 27 of the 47 men (57%) had vertebral fractures, and 16 (34%) had appendicular fractures. Causal factors identified in 30 men (64%) included glucocorticosteroid treatment (8 men); hypogonadism (7 men); excessive alcohol consumption (7 men); and anticonvulsant use, osteomalacia, severe hyperthyroidism, or bone marrow neoplasia (8 men). Seventeen men (36%) had no medical conditions or known risk factors associated with bone disease. Spinal mineral density was well below the mean value for healthy young men in 94% of the patients with primary osteoporosis tested. Examination of biopsy specimens from 13 of 17 men with primary osteoporosis showed reduced trabecular bone volumes, normal bone formation rates, and slightly increased resorption surfaces. Fasting hypercalciuria was seen in some men (41%). In the primary osteoporosis group, eight men were followed serially (range of follow-up, 6 months to 9 years) while they were receiving a nonpharmacologic regimen (diet and activity); the mean axial bone mineral density of these men increased slightly. A thorough evaluation for identifiable causes of severe osteoporosis in men is warranted because definable pathogenetic factors are seen in many cases. A few men with severe osteoporosis have primary or idiopathic osteoporosis. Primary osteoporosis in men is probably caused by many factors because heterogeneous clinical, laboratory, and histologic features were seen in our series and in those of others. Further studies of primary osteoporosis are needed to define the course of the disease, to identify pathogenetic mechanisms, and to develop therapeutic interventions.