NDUFB11 (OMIM *300403) variants have recently been independently proposed to cause histiocytoid cardiomyopathy (histiocytoid CM; OMIM 500000) (Shehata et al. 2015) and microphthalmia with linear skin defects syndrome (MLS; OMIM 309801) (van Rahden et al. 2015). Here, NDUFB11 is linked to microphthalmia.