Nevus lipomatosus cutaneous superficialis (NLCS) is a rare cutaneous hamartoma characterized by the ectopic presence of mature adipocytes in papillary and reticular dermis without connection to the subcutis. It usually has a predilection for buttocks, upper thigh and back. It presents itself as skin to yellow colored discrete or confluent papulo-nodular soft, fleshy sessile mass and has two clinical forms described in the literature, classical and solitary types. The classical form is usually composed of multiple and grouped skin-colored, pedunculated nodules. A second and more rare form is characterized by a solitary dome-shaped sessile papule or nodule. Microscopically it presents with the fat deposition in the dermis and is considered to be the consequence of the degenerative changes in the connective tissues. Its pathogenesis is unknown; however, several theories have been proposed. Some attribute it to the adipocyte metaplasia during the degenerative course and some to the development displacement of adipose tissue while others, as a consequence of maturation of adipocytes from mononuclear cells differentiation into lipoblasts in the perivascular zone. Histopathology shows clusters of ectopic presence of adipose fat among the desiccating collagen fibres in the dermis without any connection with the subcutaneous fat. Herein, we present a case of solitary NLCS over left flank associated with left upper thigh acrochordon in a 30 years female.