Spinal angiolipomas are distinct, benign lesions composed of mature lipocytes admixed with abnormal blood vessels. Three new cases of spinal angiolipoma are presented and 34 previously reported cases are analyzed. The 37 total cases (23 females and 14 males) ranged in age from 17 to 73 years (mean 43 years; median 45 years). The mean age of the female patients was older than that for the males (45.0 vs. 41.6 years; p < 0.001, Student's t-test) and most were peri- or postmenopausal. Prior to diagnosis, 97% of the patients had weakness of the lower extremities, 94% had sensory dysfunction, 84% had hyperreflexia and spasticity, 51% had sphincter dysfunction, and 41% had back pain lasting from 1 to 180 months (mean 28 months). Five (22%) of the 23 female patients were pregnant and two had exhibited significant weight gain coincident with the onset of symptoms. The angiolipomas were extradural in 35 patients and intramedullary in two; seven of the extradural lesions infiltrated the surrounding bone. The tumors extended from C-6 to L-4 and had a predilection for the midthoracic region (53% of cases). Plain radiographs were abnormal in 11 (39%) of 28 patients and in all patients with bone infiltration. Myelograms were abnormal in 97% of 32 patients and showed a complete block in 63% of patients. Computerized tomography (CT) and magnetic resonance (MR) imaging revealed the fat-density lesions in all cases studied. There was vascular enhancement in three of five cases with contrast-infused CT and in the one case with gadolinium-infused MR imaging. All patients improved following resection of the epidural lesions and internal decompression of the intramedullary lesions. It is concluded that spinal angiolipomas predominantly affect women. They involve the thoracic (especially the midthoracic) region, and produce symptoms and signs of spinal compression and, in some cases, bone erosion and pathological fractures. Their symptomatology can be exacerbated by pregnancy and weight gain, suggesting that vascular engorgement and the presence of obesity influence their evolution. Their preponderance in older, peri-, or postmenopausal women, and their clinical exacerbation in pregnant women support a role for hormonal influence. Magnetic resonance imaging is the investigation of choice for the diagnosis of these lesions. Surgery is universally successful in relieving symptoms.