Background: 5-alpha reductase deficiency is a rare etiology of 46, XY disorders of sex development (DSD), with a prevalence estimated between 1 in 100,000 to 1 in 1 million live male births. Diagnosis in resource-limited settings can be challenging due to the high cost of genetic testing. Clinical Description: The cohort consisted of 14 patients, with a mean age of 9.70 ± 5.06 years and a median age of 11 years. Six patients presented during puberty, while eight were prepubertal, including four below five years of age. Presentations varied from isolated small phallus to proximal hypospadias and cryptorchidism. Management and Outcome: Karyotyping confirmed 46 XY in all patients, and ultrasound showed no Mullerian structures. HCG stimulation test was done using standardized protocol in all patients; revealing high (>27) testosterone: dihydrotestosterone ratio. Treatment with DHT gel resulted in significant increases in phallic length, especially in younger patients. Stage-wise surgical interventions were planned for patients with varying degrees of proximal hypospadias. Conclusion: Diagnosis and management of 5-alpha reductase deficiency in resource-limited settings can be effectively approached using clinical evaluation, hormonal assays, and imaging studies. HCG-stimulated testosterone to DHT ratio is a useful diagnostic tool, with higher cut-offs may improve specificity in the absence of genetic testing. DHT gel treatment significantly improves phallic length, demonstrating its efficacy in managing this condition.