Affecting only 1 in 200,000–300,000 persons worldwide, epidermolytic ichthyosis (EI) is a rare autosomal dominant genodermatosis presenting with a characteristic phenotype of generalized corrugated scaling. We report a case of EI highlighting significant improvement in clinical outcomes and quality of life. A 27-year-old Filipino male sought to consult for generalized foul-smelling “wood bark” like scaling since early childhood. Histologic findings confirmed EI. The condition was managed with hydration, lubrication, keratolysis, and low-dose isotretinoin (0.5 mg/kg/day). Clinical outcomes, measured using the visual index for ichthyosis severity scoring and dermatology life quality index, revealed significant improvement. EI is a rare life-long disease of cornification which can severely incapacitate the person affected. Given the limited cases, this case report supports the efficacy of oral isotretinoin in its treatment. The recovery in the cosmetic and functional capacity parallels the upliftment of psychosocial burden. Overall, the management led to an improvement in the quality of life.