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Background: Cardiac disorders are found in about half of cases of Noonan syndrome (NS). The most common congenital heart diseases in this syndrome include pulmonary valvular stenosis obstructive or nonobstructive hypertrophic cardiomyopathy (17%). Biventricular hypertrophic cardiomyopathy (HCM) is very rare in this condition., Objective: The objective is to report a case of biventricular hypertrophic cardiomyopathy in a 26-year-old Nigerian female with the phenotype., Methods: This is a descriptive case report., Results: The patient presented with dyspnoea on exertion which started at the age of 7 years and has progressively worsened. There was associated precordial chest pain and palpitation. Clinical examination revealed a young woman, who is small for her age. She had some dysmorphic features such as a webbed neck, lowset ears, low posterior hairline, crowded teeth, high arched palate, a small and asymmetric chin and a high carrying angle at the elbows. The pulses were synchronous and there was no radio-radial or radiofemoral delay and her blood pressures were within normal limits. Cardiac auscultation was unremarkable. The 12-lead ECG showed biventricular hypertrophy with a strain pattern. The echocardiogram showed features in keeping with biventricular hypertrophic cardiomyopathy., Conclusion: Biventricular HCM is relatively uncommon in Noonan syndrome. Patients with typical dysmorphia should have a full cardiac evaluation to look for these anomalies., Competing Interests: The Authors declare that no competing interest exists, (Copyright © 2024 by West African Journal of Medicine.)