Two different recessive forms have been described: (1) the NLSD with myopathy (NLSD-M) caused by molecular defects in the adipose triglyceride lipase gene (ATGL, also called patatin-like phospholipase domain-containing 2, PNPLA2) coding for a rate-limiting enzyme catalyzing the first step of hydrolysis of triglycerides [1], and (2) the NLSD with ichthyosis (NLSD-I or Chanarin-Dorfman Disease) due to mutations in the ABHD5 gene (also known as Comparative Gene Identification-58, CGI-58) coding an homonymous activator protein of ATGL [2]. The gene discussed is PNPLA2; the disease is ichthyosis.