GRIM19 codes for an effector of apoptosis, and is also part of the mitochondrial Complex I. As several new missense variants in GRIM19 were identified in sporadic cases and in two families with multiple affected individuals presenting oncocytic tumours (Maximo et al, 2005), this gene was screened in a larger panel of multigenerational families such as the ones we collected. The gene discussed is NDUFA13; the disease is oncocytic neoplasm.