We report a case of a two and a half yr old boy who presented with complaint of bilateral asymmetrical breast enlargement since infancy. On examination, he had features of neurofibromatosis type 1 (NF1). Complete endocrinological evaluation was normal. Trucut biopsy of the breast revealed overgrowth of fibrocollagenous and adipose tissue without hyperplasia of breast parenchyma. Thus a diagnosis of NF1 with pseudogynecomastia was made.