Germline SMARCE1 heterozygous loss‐of‐function variants have been found in young patients with cranial and spinal meningiomas, consistent with a tumor suppressor mechanism (Lomelí & Castillo‐Robles, 2016; Smith et al., 2014) while missense variants cause Coffin–Siris syndrome (Kosho & Okamoto, 2014). Here, SMARCE1 is linked to Coffin-Siris syndrome.