The lipomas of the central nervous system are rare lesions of congenital origin and are located in the medial line and especially in corpus callosum. Intramedullary spinal lipomas can be seen in the life span of 30 years of age and most frequently coincide with initial puberty period. Magnetic resonance imaging and computerized tomography together with clinical trials are of crucial importance for diagnosis. The first case: A two-year-aged girl who had lipoma in quadrigeminal cistern and who suffered from encephalocraniocutaneous lipomatosis has been clinically studied. The fascial lipoma of the patient has been excised subtotally by the Plastic Surgeons; then the patient has underwent supracerebellar infratentorial operation where the intracranial lipoma has been excised by our team. The histopathology has been reported to be consisting of peripheric nerve tissue and calcification. The second case: A twenty-year-aged man with intramedullary lipoma localized between T1-T4 has been given our clinical trials. T1-T5 total laminectomy and subtotal excision were made for this patient. Due to the fact that the lipomas of central nervous system are rarely seen and are involved in nervous and calcific tissues except for fatty tissues they can be mistaken for hamartomatous masses. The total excision of the lipomas of central nervous system and especially the spinal intramedullary lipomas are quite difficult to be excised since they are tightly entangled with the neural tissue. So any attempt for total excision would be dangerous. Operation for decompression and biopsy is of primary concern.