A 31-year-old woman with Netherton syndrome diagnosed in childhood was referred to our clinic for evaluation and treatment of an atrophic defect of the right ala of the nose. The patient reported that the lesion began to develop with a small scab approximately four weeks ago and developed into an expanding, painless ulcer. She denied trauma and factitious manipulation. Patient history included previous hospital admissions for systemic and topical treatment of the underlying disease, and a consanguin marriage in former generations. On clinical examination the patient’s skin was erythro-dermic, xerotic and scaling. On palpation, however, the skin-soft tissue envelope appeared soft and pliable. The patient had chapped and rough lips, easily starting to bleed when opening the mouth. Moreover she had dry, brittle and bristly hair and almost no eyebrows and lashes. There was an endophytic 4 millimeter ulcer of the right nasal ala as shown in Figure. Endoscopic rhinoscopy revealed the full thickness defect, but otherwise unremarkable mucosa within the nasal cavity. The histologic evaluation of a biopsy from the edge of the defect showed a well-differentiated squamous cell carcinoma. Ultrasonography of the neck revealed no suspicious lymph nodes. The tumor was resected under local anaesthesia, minimal margin was 4 mm on permanent histologic section. In terms of reconstruction, we discussed with the patient various options including single – stage placement of a composite graft, and a staged reconstruction with an epidermal turn-in flap and a paramedian forehead flap with the option of a third stage thinning of the flap, if needed. The patient opted for the staged approach. In the first stage, inner lining was reconstructed with two epidermal turn-in flaps of the right nasal sidewall. The skin texture and thickness appeared not ideally suited for the transposition of a nasolabial flap, and hence a paramedian forehead flap, based on the supratrochlear artery was selected for epithelial closure. The donor defect of the forehead was closed primarily. Pedicle division and contouring of the flap was performed in the secondary constructive stage 3 weeks later under local anaesthesia. Wound healing was unremarkable. The patient has remained free of disease for 11 months follow up.