Nevus Lipomatosus Cutaneous Superficialis (NLCS) is an uncommon hamartomatous lesion of the skin. NLCS is classified into two clinical forms classic and solitary. Some authors consider this lesion as a type of connective tissue nevus. We report a rare case of solitary NLCS in a 69 year old male with a long history of pedunculated swelling in the right gluteal cleft with review of literature. On gross appearance the lesion was a grey brown, exophytic, cerebriform, smooth skin covered soft tissue mass measuring 4.5 cms in the largest dimension with a broad peduncle attached. On histopathological examination the lesion showed superficial and deep dermal infiltration of the adipose tissue with distortion of the dermal collagen.