Alar rim defects are most commonly acquired as a result of trauma, burns, tumor excision or sometimes accompanying craniofacial clefts. However, isolated congenital alar defects are extremely rare occurring in about 1 in 20,000 to 40,000 live births. We are presenting a case report of an isolated congenital cleft of the alar rim. The defect was closed by the use of a rotation advancement full-thickness flap. With this technique, both symmetry and desired thickness of the nostrils were achieved. The skin color and texture of the alar rim were good with minimal scars.