Large cell calcifying sertoli cell tumor of the testis is an uncommon variant of sertoli cell tumors, accounting for less than 1% of all testicular neoplasms. Although it is usually benign and nmltifocal, malignant forms also exist but are exceptional. The tumor is predominantly associated with hereditary endocrine anomalies like Carney complex (myxomas of the heart, skin, oral cavity and breast, adrenocortical hyperplasia, pituitary adenomas, lentigines of the skin and other anomalies) and Peutz-Jeghers syndrome. A young child with a testicular mass that proved to be a large cell calcifying sertoli cell tumor is reported here. We discuss the pathological and clinical findings in terms of their correlation after a 2-year follow-up of the patient.