NHLRC2 (NHL repeat containing protein 2) is an essential cytosolic protein of unknown function [1], mutation of which was recently identified in association with FINCA (fibrosis, neurodegeneration, cerebral angiomatosis) disease, a multi-organ condition which is fatal in early childhood [2]. Here, NHLRC2 is linked to fibrosis, neurodegeneration, and cerebral angiomatosis.