Introduction: Dyshormonogenetic goiter is a rare inherited disease caused by defects in thyroid hormones synthesis. It mainly affects children and young adults. It is frequently associated with congenital hypothyroidism. Objective: The aim of our study is to highlight the clinico-pathological characteristics of dyshormonogenetic goiter with a review of literature. Methods: we reported 4 cases of dyshormonogenetic goiter over a period of 25 years. Results: There were 2 male and 2 female patients. The mean age was 16 years. All patients were treated with partial or total thyroidectomy and L-thyroxine. Long term follow-up showed relapse in one case and no complications in three cases. Conclusion: The diagnosis of dyshormonogenetic goiter is suspected in clinical and radiological features and confirmed by histological examination.