OBJECTIVES: Results from a comprehensive literature review of hutchinson-gilford progeria syndrome (HGPS): more research is needed? METHODS: Information was retrieved from websites of World Health Organization, Pubmed, clinicaltrials.gov and relevant grey literature using key words like Progeria, HGPS etc. Data regarding prevalence rate and current treatment options were obtained and complied. RESULTS: Progeria is an extremely uncommon genetic condition (ICD-10.E34.8), wherein symptoms resembling aspects of aging are manifested at early age. To our knowledge, only four clinical trials are in-process and results are waiting. No randomized clinical trial has been reported till date. The prevalence rate of Progeria is approximately 1 per 4 million births. A total of 150 cases have been reported since 1886 and 40 cases are living currently. The p.G608G LMNA mutation is the most commonly reported mutation. Around 95% of patients are Caucasians and male to female ratio is anticipated as 1.5:1. Symptoms of the disease start appearing at an age of 6-12 months. The patients demonstrate a life expectancy of average 13 years (range: 7-27 years). Death largely occurs due to cardiovascular abnormalities. New treatment options include Farnesyltransferase inhibitor (FTI) (ABT-100) which is presently under clinical study conducted by Progeria Research Foundation (PRF). FTI has shown positive results in previous studies on animals. Clinical trials are in process evaluating Lonafarnib, Zoledronic Acid, and Pravastatin in retarding the process of aging in Progeria patients. Growth hormone treatment in a few patients has also shown growth in height by 10% and in weight by 50%. Improvement in joint stiffness and BMR has been observed with Physical therapy and occupational therapy. CONCLUSIONS: The pace of research in this area has increased in recent years. Extensive genetic research can play a vital role in the early treatment of this disease.