Objectives Rare disease term has been used first time by Bull in 1878. High price tag of orphan medicines make them more attractive to the pharmaceutical companies which are loosing patent of blockbusters. Pharma companies started to invest in rare disease area to find new treatments in last decades. The aiim of the present analysis is to understand the trend of orphan medicines and rare diseases. Method A computer based data search has been conducted. Published studies were searched in PubMed with “orphan medicines or rare diseases” from 1997 to 2016. Published studies were searched with “rare diseases” key word from 1997 to 2016 in ISPOR Scientific Presentations Database. Total approved medicines number and new approved medicines number were searched in European Medicines Agency database from 2012 to 2016. Descriptive analysis have been conducted with the retrieved data. Results A total of 10816 studies were published between 1997 to 2016 in PubMed. Compaund Annual growth rate(CAGR) for published articles was calculated as 23,15%. First study was published in the ISPOR 9^th International Annual Meeting, May 2004. Number of published studies increased each year with 38.77% CAGR. A total of 183 studies were published in ISPOR meetings. Approved orphan medicines were increased from 13 to 81 from 1997 to 2016 with a 8,05% CAGR. Conclusion Published studies and approved new molecules have been increasing in each year. Rare disease and orphan medicine areas are focused topics for pharmaceutical companies and researchers in last decades. While the first published article in PubMed was in 1878, the first pharmacoeconomic study was published in ISPOR meetings in 2004. We conclude that the pricing and reimbursement of orphan medicines have been important for decision makers since 2004. On the other hand, more new molecules might be approved in following years compared to the last decades.