This study reports two female patients with progressive muscular rigidity beginning to the paravertebral and pelvic muscles associated with muscular spasms. The electromyography showed continuous muscular activity originated at the central nervous system. In both cases we observed the complete rigid man syndrome. Complementary explorations including thyroid hormonal study, ceruloplasmin, electroencephalogram, cranial computerized tomography, and magnetic resonance were normal. Auditive evoked potentials evidenced a prolongation of the interlatency III-V, suggesting pathologic involvement of the encephalic trunk. In one patient an artificial reservoir was inserted to instill baclofen into the meningeal space and produced a clinical improvement. The actual status of this rare illness is reviewed.