Introduction: Precocious means developing unusually early .Precocious puberty is onset of puberty before 8 year in girls and 9 year in boys.¹ When it is because of premature activation of hypothalamic pituitary axis, it is called central or complete precocious puberty. If it results from ectopic gonadotropin secretion, it is called peripheral precocious puberty. We hereby present a two year old menstruating girl with precocious puberty as a rare presentation.

Case Presentation: A two year old baby girl was brought by her parents to Gynecology outpatient department with complaint of per vaginal bleeding. On examination, her pubic and   axillary hair, and  breast were developed with Tanner stage 2 and 4 respectively. Her routine blood investigations and hormone profile were normal except increased level of Dehydroepiandrosterone. X-ray of her distal radio-ulnar joint and MRI scan of her brain were normal. On ultrasonography, a mass was visible (5.5×5.4 cm) in left adrenal gland which was confirmed by CT scan (5.2×3.9cm).

 Conclusion: Hereby, we report an extremely rare case of peripheral precocious puberty with Tanner Stage 4 breast development and pubic hair (Tanner Stage 2) in a menstruating two year old girl due to benign adrenal adenoma.

Key words: Precocious puberty, Pseudo-precocious puberty, Peripheral precocious puberty, Adrenal adenoma.