Objective: This report presents an extremely rare case of heminasal aplasia or hemi-arhinia with only about 80 cases reported so far. The exact etiology and mechanism of development of heminasal aplasia are still unknown. Furthermore, the rarity of this anomaly makes its reconstruction a surgical challenge with a diversity and controversy on the timing and technique of such reconstructive procedures.

Methods: Reconstruction was performed when the child was 3 months of age. The missing heminose was reconstructed by a superiorly based nasolabial flap.

Results: On early follow-up visits, there was retraction of the flap. However, the parents were satisfied by the result to the degree that the girl did not show for follow up after 3 years of surgery until she is 13 years and even now her parents do not want any further surgery.

Conclusions: Early surgical reconstruction is recommended even if revisions may be needed later.