Membranous lipodystrophy, an uncommon disorder, was described by Nasu et al. in 1973 as a form of regressive degeneration or localized destruction of the adipose tissue. Clinical features of the lesions with membranous fat necrosis are variable and the condition can only be diagnosed histopathologically. These peculiar changes in fat tissue have been associated with many local and systemic diseases including lupus erythematosus, diabetes mellitus, erythema nodosum, stasis dermatitis, morphoea and trauma, but occasionally no underlying disease is found. Even though various hypotheses concerning the pathogenesis of membranous lipodystrophy have been proposed, the exact causes are still in dispute. We reviewed 22 patients having membranous lipodystrophy confirmed by biopsy and discuss the pathogenetic mechanisms which have been suggested by many authors.