It is very difficult to make EBM recommendations on rare and uncommon cases especially because of the paucity of published studies.
Therefore you could generally not determine if management A is better than the B based on classical EBM approach (the chances there is 1 or more RCT on an intervention are low). Therefore the quality of evidence would be downgraded based on the type of study the evidence is based on.
There are several papers on that topic:
Day S. Evidence-based medicine and rare diseases. Adv Exp Med Biol. 2010;686:41-53. doi: 10.1007/978-90-481-9485-8_3. PMID: 20824438.
Kruer MC, Steiner RD. The role of evidence-based medicine and clinical trials in rare genetic disorders. Clin Genet. 2008 Sep;74(3):197-207. doi: 10.1111/j.1399-0004.2008.01041.x. Epub 2008 Jul 23. PMID: 18657147.
If you are looking for results, procedures to follow or appropriate care, the best thing to do is to use the internationally recognized site: ORPHANET, which is promoted in the European member states.
If you want to adopt an EBM approach when you are faced with a rare case, you could follow the recommendations of CARE guidelines (https://www.care-statement.org/case-reports). You can find tools on : https://www.swihm.com/
In the case of rare diseases, colleagues are carrying out meta-analyses of case-control studies. You can find an example here :
Sampayo-Cordero, M., Miguel-Huguet, B., Pardo-Mateos, A. et al. Agreement between results of meta-analyses from case reports and clinical studies, regarding efficacy and safety of idursulfase therapy in patients with mucopolysaccharidosis type II (MPS-II). A new tool for evidence-based medicine in rare diseases. Orphanet J Rare Dis 14, 230 (2019). https://doi.org/10.1186/s13023-019-1202-6