<prompt> The most likely diagnosis is a case of the intractable convulsions mental defect and facial nevus is-Sturge Weber syndromeTuberous sclerosisVon-Hippen -Lindau diseaseVon- Reckling-Hausens disease  <response>: Sturge Weber syndrome and Von-Reckling-Hausen disease NF-1 have been explained earlier about other options. Tuberous sclerosis Bournevilles diseaseTuberous sclerosis is characterized by : -Cutaneous lesions Adenoma sebaceum, ash-leaf shaped hypopigmented macules, shagreen patches, depigmented nevi.SeizuresMental retardationOther features Calcified subependymal nodules, Subependymal giant cell astrocytoma, ependymoma,Rhabdomyomas of the heart, and angiomyomas of the kidney, liver, adrenals and pancreas.Von Hippel - Lindau syndromeThis syndrome consists of Retinal, cerebellar and spinal hemangioblastoma.Renal cell carcinoma, Pheochromocytoma, benign cyst of the kidney, liver, pancreas and epididymis may also occur.Polycythemia may occur due to secretion of erythropoietin from hemangioblastoma. 