The authors report a case of cervical intramedullary lipoma extending into the bulbomedullary junction. 99 cases of cervical and/or dorsal intramedullary lipomas without spinal dysraphism, reported since 1884, are reviewed. There is poor correlation between lipoma's length and age of onset. Only lipoma extended to all the spinal cord begin in the first years of life. Clinical presentation is in 58% of cases a slowly compressive myelopathy, a syringomyelic syndrome in 9.5% of cases, a Brown Sequard syndrome in 6.5% of cases or atypical clinical features in 26% of patients. Finding of a subcutaneous lipoma at the level of the lesion helps for diagnosis. C.T. scan and magnetic resonance imaging (M.R.I.) precise lipomatous constitution of the tumor, its limits and relations with neighbouring tissues. Surgery is necessary when clinical features are advanced. Surgery indication is debatable when neurological manifestations are poor or absent. Post operative course is generally good and is not related with type of intervention.